Agarwal - Manual of Neuro Ophthalmogy

Manual of
Neuro-ophthalmology
Manual of
Neuro-ophthalmology
Amar Agarwal MS FRCS FRCOphth

Athiya Agarwal MD DO
Dr Agarwals Group of Eye Hospitals and Eye Research Centre
19, Cathedral Road, Chennai - 600 086, India
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Manual of Neuro-ophthalmology
2008, Jaypee Brothers Medical Publishers
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are to be settled under Delhi jurisdiction only.
First Edition: 2009
ISBN 978-81-8448-411-3
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This book is dedicated
to a lovely couple
Marguerite Mcdonald and Stephen Klyce
Contributors
Amar Agarwal MS FRCS FRCOPHTH

Dr. Agarwals Group of Eye Hospitals
and Eye Research Centre
19, Cathedral Road
Chennai-600 086, India
dragarwal@vsnl.com
Athiya Agarwal MD DO
Dr. Agarwals Group of Eye Hospitals
and Eye Research Centre
19, Cathedral Road
Chennai-600 086, India
dragarwal@Vsnl.com
Garrett Smith MD
Moran Eye Center
Salt Nake City, UTAH
USA
Jeyalakshmi Govindan DO DNB

Consultant Ophthalmologist
Dr. Agarwals Eye Hospital
19, Cathedral Road
Chennai, India
Nick Mamalis MD
Moran Eye Center
Salt Nake City, UTAH
USA
P Ramesh MBBS DMRD DNB MNAMS FRCR

Director, Liberty Scans
Chennai, India
Priya Narang MS
Narang Eye Hospital
Ahmedabad, Gujarat, India
viii Manual of Neuro-ophthalmology
Reena M Choudhry MD DO DNB FRCS

Icare Eye Hospital and Postgraduate Institute
Noida, Uttar Pradesh
India
Sameer Narang MS
Narang Eye Hospital
Ahmedabad, Gujarat
India
Saurabh Choudhry MD DO DNB

Icare Eye Hospital and Postgraduate Institute
Noida, Uttar Pradesh
India
S Soundari DO DNB FRCS

Consultant Ophthalmologist
Dr. Agarwals Eye Hospital
19, Cathedral Road
Chennai
India
Foreword
Neuro-ophthalmology is a complex subspecialty which requires keen

skills of clinical observation, attention to detail, and intricate thought
processes in order to formulate the appropriate diagnostic and
therapeutic plan for the patient. What makes the field even more
challenging is our limited knowledge of the intricate neurological
pathways between the eye and the brain; many of which are still
being discovered, as long as our understanding is evolving.
To concisely and accurately explain the basics of neuro-
ophthalmology is a difficult task, as it requires a thorough
understanding of the subject as well as a natural gift for simplifying
and organizing the material so that it appeals to a wide audience.
Through the process of teamwork, the Agarwals have succeeded in
creating an outstanding book for neuro-ophthalmology that will prove
to be an excellent reference for a full spectrum of readers, from medical
students to practising ophthalmologists.
Prof Amar Agarwal once explained to me that for any challenging
situation, The battle is in the brain. Whether the task is climbing
Mount Everest or writing a complete library of ophthalmology texts,
the true challenge is in mind. Having the drive and determination to
succeed, no matter the situation, is the mark of a true pioneer, and a
characteristic of one of my strongest mentors, Prof Amar Agarwal.
Uday Devgan MD FACS

Chief of Ophthalmology
Olive View-UCLA Medical Center
UCLA School of Medicine
Private Ophthalmic Practice
Maloney Vision Institute
Los Angeles, California, USA
Preface
Understanding Neuro-ophthalmology is a challenge. It took us a long

time to comprehend the basics in this field when we were residents.
That is the notion why we have written Manual of Neuro-ophthalmology.
The idea is that you dear reader can go through the text and figures
and never have difficulty in understanding this subject like we did.
We would like to thank our consultant Dr S Soundari for helping
us. Shri JP Vij and his full team of M/s Jaypee Brothers Medical
Publishers have always supported our writing endeavors. Our sincere
thanks to them. Finally, dear reader we hope this book will change
your outlook to Neuro-ophthalmology.
Amar Agarwal
Athiya Agarwal
Contents
1. Supranuclear Pathways for Eye Movements ..................................... 1
Athiya Agarwal, Amar Agarwal
2. Supranuclear Disorders of Eye Movements ....................................17
3. Nystagmus .............................................................................................32
4. The Pupil ................................................................................................54
5. Visual Pathway .....................................................................................73
Athiya Agarwal
6. Anatomy of the Optic Nerve ............................................................ 103
Athiya Agarwal
7. Oculomotor Nerve ............................................................................. 109
Athiya Agarwal
8. Lesions of the Oculomotor Nerve ................................................... 118
Athiya Agarwal
9. Trochlear Nerve and its Lesions ..................................................... 123
Athiya Agarwal
10. Abducent Nerve and its Lesions ..................................................... 132
Athiya Agarwal
11. Trigeminal Nerve .............................................................................. 140
Athiya Agarwal
12. Facial Nerve and its Lesions ............................................................ 145
Athiya Agarwal
13. Congenital Optic Nerve Anomalies ................................................ 150
Priya Narang, Sameer Narang, Amar Agarwal
14. Optic Nerve Tumors ......................................................................... 157
Nick Mamalis, Garrett Smith
15. Abnormalities of Optic Nerve Head .............................................. 185
Reena M Choudhry, Saurabh Choudhry, Amar Agarwal
16. Ocular Myopathies ............................................................................ 197
S Soundari
17. Miscellaneous .................................................................................... 204
Jeyalakshmi Govindan, S Soundari
18. Examination of a Neuro-ophthalmology Case .............................. 219
S Soundari
19. Imaging in Neuro-ophthalmology .................................................. 226
P Ramesh
Index ..................................................................................................... 253
Supranuclear
1 Pathways for Eye
Movements
INTRODUCTION
One is always confused about supranuclear pathways. We understand
the pathways of the III, IV and VI cranial nerve nuclei. We would be
able to trace it from the brain to the superior orbital fissure, but we
fail to remember that these pathways we are discussing are the
infranuclear pathways which extend from the cranial nerve nuclei to
the ocular muscle. We need to also understand the anatomy of the
supranuclear pathways.1,2
SUPRANUCLEAR AND INFRANUCLEAR PATHWAYS

Anatomical pathways, which extend from the cortical centers of the
brain to the cranial nerve nuclei, are called the supranuclear pathways.
From the cranial nerve nuclei to the ocular muscle exist the infranuclear
pathways (Fig. 1.1).
In peripheral nerves, the nerve starts from the brain and reaches
the anterior horn cell in the spinal cord. This is the upper motor
neuron. From the anterior horn cell of the spinal cord, the nerve moves
to the peripheral muscle. This is the lower motor neuron. If there is a
lower motor neuron disease the limb is flaccid and if there is an upper
motor neuron disease the limb is spastic.
The cranial nerve nuclei are like peripheral nerve nuclei. From the
cortex of the brain the nerve extends to the cranial nerve nuclei and
this is the upper motor neuron (UMN) pathway. From the cranial
nerve nuclei the nerve extends to the ocular muscle and this is the
lower motor neuron (LMN) pathway. In peripheral nerves if the
anterior horn cell gets involved as in poliomyelitis, the patient has a
LMN disease and so the limb is flaccid. The anterior horn cell is akin
to the cranial nerve nuclei of cranial nerves. So, if the cranial nerve
nuclei gets involved the lesion produced will be a LMN lesion.
2 Manual of Neuro-ophthalmology
Fig. 1.1: Supranuclear pathway
SUPRANUCLEAR EYE MOVEMENT SYSTEMS

There are five supranuclear eye movement systems. They are:
1. Saccadic system
2. Pursuit system
3. Vergence system
4. Non-optic reflex system
5. Position maintenance system.
SACCADIC SYSTEM
The saccadic system is otherwise known as the fast eye movement
system or rapid eye movement system. This is because the saccadic
system controls the fast eye movements. These are command
movements. For example if we say, look to the right, the eyes turn to
the right. This occurs rapidly and is a rapid eye movement. The system,
which controls this command pathway, is the saccadic system.
The saccadic system originates from the frontal lobe of the brain.
The impulses then move to the mesencephalic system and so the
anatomical pathway subserving the fast eye movements is the
Supranuclear Pathways for Eye Movements 3
frontomesencephalic pathway. When you watch someone watching a game

of tennis or table tennis, you will notice the eyes move rapidly from
one end of the court or table to the other. The eyes keep on darting
from one end to the other. These are fast eye movements controlled
by the frontomesencephalic pathway.
Horizontal Saccades
The saccades can in turn be horizontal or vertical. In horizontal
saccades, the eyes move horizontally and in vertical saccades, the
eyes move up and down. Let us now understand the pathway of the
horizontal saccades (Fig. 1.2).
Fig. 1.2: Horizontal saccade pathway

LR- Lateral rectus; MR- Medial rectus; LE- Left eye; RE- Right eye; Occ.Lobe-
Occipital Lobe; Fron.lobe- Frontal lobe; III- III Cranial nerve nucleus; VI- VI Cranial
nerve nucleus; PGC- Pontine gaze center; MLF- Medial longitudinal fasciculus;
UMN Pathway- Upper motor neuron pathway; LMN Pathway- Lower motor neuron
pathway
If the eyes have to look to the right, then the command for this
movement is given by the left frontal lobe in area 8 of the cortex. The
nerves cross over to the opposite side and reach the right pontine
gaze center. From here the nerves pass to the same side (in this case
the right) VI nerve nuclei. From the right pontine gaze center nerves
also pass to the opposite III nerve nuclei. In this case this will be the
left III nerve nuclei. All the cranial nerve nuclei are connected with
each other through the medial longitudinal fasciculus or medial
longitudinal bundle. In other words from the right pontine gaze center,
the nerves pass through the medial longitudinal bundle to the left III
cranial nerve nuclei. Till here is the supranuclear pathway. This is
why this is also called the frontomesencephalic pathway.
From the right VI nerve nucleus nerves then pass to the lateral
rectus muscle of the right eye. From the left III nerve nucleus nerves
pass to the left medial rectus muscle. These are the infranuclear
pathways and both the eyes move to the right.
At this stage it is important to understand a bit more on the
medial longitudinal bundle. As just explained, the nerves pass from
the pontine gaze center to the VI and III nerve nuclei through the
medial longitudinal bundle. If there is a lesion in the medial
longitudinal bundle, these fibers are cut and there would not be a
correlation between the III nerve and the VI nerve. This leads to the
condition called internuclear ophthalmoplegia.
Vertical Saccades
The pathway for the vertical saccades is still doubtful. Vertical saccades
depend on simultaneous bilateral activity within the frontal lobes in
Area 8 (Fig. 1.3). This means that the horizontal saccades are
unilaterally controlled whereas the vertical saccades are bilaterally
controlled.
If one has to look up or down, impulses travel from both the frontal
lobes in Area 8. The impulse travels via the basal ganglia to the pretectal
area or the pretectal center for vertical gaze. This is the vertical gaze
center. From the vertical gaze center impulses pass to the III nerve
nuclei. Till here is the supranuclear pathway. Now, the infranuclear
pathway starts and impulses go via the III cranial nerve to the vertical
muscles and the patient looks up or looks down.
Because of the fact that vertical saccades require bilateral cortical
activity, cerebral hemisphere lesions rarely produce deficits in the
vertical saccades. Such deficits are seen only with massive hemispheric
lesions producing bilateral damage to both frontomesencephalic
Fig. 1.3: Vertical saccade pathway

LE- Left eye; RE- Right eye; Occ.Lobe- Occipital Lobe; III- III Cranial nerve nucleus;
VI- VI Cranial nerve nucleus; PGC- Pontine gaze center; MLF- Medial longitudinal
fasciculus; UMN Pathway- Upper motor neuron pathway; LMN Pathway- Lower
motor neuron pathway
pathways. Disturbances of vertical saccades are much more common

with midbrain disorders.
Characteristic of the Saccade

The characteristic of the saccades is shown in Table 1.1 compared to
the other supranuclear eye movements. From the onset of the stimulus,
which is voluntary to the beginning of the recorded saccade, the latent
period is about 200 to 250 msec. The velocity of the fast eye movement
is 30 to 700 degrees/second.
PURSUIT SYSTEM
The smooth pursuit system is utilized when the eyes follow targets
that move smoothly and relatively slowly. It maintains a fixed
relationship between the movements of the eyes and the target. As
smooth pursuit movements directly relate eye position to target
position, they are also termed as following or tracking movements. As
these movements are slow, they are called slow eye movements. Imagine
a person walking and you are watching that person. When your eyes
follow the movement of the person, they will be using the pursuit
system. The pathway for the pursuit system starts from the occipital
lobe and hence is known as the occipitomesencephalic pathway. There
are different pathways for horizontal pursuits and for vertical pursuits.
Horizontal Pursuit System Pathway

If a target is moving to the right (Fig. 1.4), the first step is that the
eyes have to visualize the object. So the pathway starts from the retina
Fig. 1.4: Horizontal pursuit pathway (slow phase)

pathway
of both eyes. The impulses pass through the optic nerve, optic chiasma,
and optic tract and reach the right occipital lobe in area 19. This area
subserves the pursuit movements. It is important to note that the
occipital areas mediate horizontal pursuit movements to the ipsilateral
side. In other words, the right occipital lobe mediates horizontal
pursuit movements to the right.
From the occipital lobe, impulses go to the same side pontine gaze
center. In this case, impulses from the right occipital lobe go to the
right Pontine gaze center. From here impulses go to the right VI nerve
nucleus and the left III nerve nucleus. Till here is the supranuclear
pathway. From the right VI nerve nucleus and the left III nerve nucleus
impulses go via the infranuclear pathway to the lateral rectus and the
medial rectus. The characteristics of the pursuits are shown in
Table 1.1.
Corrective Saccade
When the target is moving away from the field of vision the eyes
which were moving slowly to that side have to come back to their
original position. A fast eye movement does this, in other words a
saccade. This is the corrective saccade. If a stream of cars are going in
front of our vision, then we keep on following one car and when it
goes out of the field of vision our eyes would come and fixate back to
the car in the center of our field of vision. This would be done by the
corrective saccade.
As the impulses from the target moving to the right reaches the
occipital lobe (Area 19) and the object is going out of the field of
Table 1.1: Characteristics of eye movements

Type Stimulus Latency Velocity Amplitude Conjugacy
(msec) (Deg./Sec) (Degrees)
1. Saccade Volition, reflex 200 30-700 0.5-9.0 Conjugate
2. Pursuit Target motion 125 < 50 0-90 Conjugate
3. Vergence Accommodative, 160 < 20 Age Disjugate
fusional dependent
4. Vestibulo- Head movement <100 < 400 0-90 Conjugate
ocular
5. Corrective Positions error 125 < 150 <4 Conjugate
saccade
6. Micro- Fixation - 3-12 1-25 Conjugate
saccade
7. Microdrift Fixation - 0-30 <1 Disjugate
min/sec
vision the occipital lobe sends impulses to the ipsilateral frontal lobe
to perform the corrective saccade. In this case the right occipital lobe
(Fig. 1.5) sends impulses to the right frontal lobe (Area 8). This means
there has to be a communication between the occipital lobe and the
frontal lobe. From the right occipital lobe impulses pass to the frontal
lobe via the parietal lobe.
From the right frontal lobe, impulses then pass to the left pontine
gaze center which in turn sends impulses to the left VI nerve nucleus
and the right III nerve nucleus. This is the supranuclear pathway.
Then, the infranuclear pathway takes over and impulses go to the
respective lateral and medial recti and the eyes move to the left as a
fast eye movement. This is the corrective saccade.
Fig. 1.5: Corrective saccade (Horizontal pursuit pathway for the fast phase)
LR- lateral rectus; MR- Medial rectus; LE- Left eye; RE- Right eye; Occ.Lobe-
pathway
One can illustrate this with an optokinetic drum, which is a drum

with black and white stripes. The drum is rotated and the eyes fixate
on it. When the stripes go away from the field of vision, the corrective
saccade occurs. This leads to a type of nystagmus known as opto-
kinetic nystagmus.
Parietal Lobe Lesion

If the person has a parietal lobe lesion, then there is a problem (Fig.
1.6). When the corrective saccade has to work the impulse would not
pass beyond the parietal lobe. Thus, this would lead to a deficit in the
corrective saccade. So a deep parietal lobe lesion causes loss or decrease
of the fast phase of the optokinetic nystagmus, when movement of
the drum is towards the side of the lesion.
Fig. 1.6: Parietal lobe lesion

UMN Pathway- Upper motor neuron pathway
Vertical Pursuit System

Vertical pursuit movements are generated by simultaneous bilateral
stimulation of area 19 of the occipital lobe (Fig. 1.7). The axons of the
occipital lobe descend to the pretectal area. From the pretectal area
impulses travel to the III nerve nuclei. Till here is the supranuclear or
UMN pathway. Then from the III nerve nuclei, impulses pass to the
vertical muscles via the infranuclear pathway. The pretectal area or
pretectal center is the center for vertical gaze, analogous to the pontine
gaze center, which is the center for horizontal gazes.
Fig. 1.7: Vertical pursuit pathway

LE- Left eye; RE- Right eye; III- III Cranial nerve nucleus; VI- VI Cranial nerve
nucleus; PGC- Pontine gaze center; MLF- Medial longitudinal fasciculus; UMN
Pathway- Upper motor neuron pathway; LMN Pathway- Lower motor neuron
pathway
VERGENCE SYSTEM
The role of the vergence system is to keep the image of a target on
appropriate points (corresponding elements) of the two retinas by
controlling the visual axes of the eyes. Thus, vergence is utilized
whenever a target falls on noncorresponding retinal elements. For
example, if a target is moved towards the eyes, they must turn toward
each other (converge) to keep the target on the fovea of each eye.
Conversely, as the target is moved further away, the eyes must turn
out (diverge) (Actually, divergence does not occur in our eyes.)
Vergence is thus a disconjugate (nonparallel) movement of the eyes,
in contrast to most other eye movements which are conjugate (parallel).
There are two types of vergence. They can be voluntarywhen we
command our eyes to converge or reflexwhen we bring an object
or tape towards our nose and the eyes converge while fixating on the
object. The characteristics of the vergence movements are shown in
Table 1.1.
Voluntary Vergence
The center for voluntary vergence is situated in area 8 of the frontal
lobe (Fig. 1.8). If one wants to converge then a command movement
is sent from area 8. These are bilateral impulses and they go to the
pretectal area via the basal ganglia. Here there is the convergence
area. From the convergence area, impulses go bilaterally to the III
and VI nerve nuclei. Till here is the supranuclear pathway. From the
III nerve nuclei impulses go to the medial recti to converge. From the
VI nerve nuclei inhibitory impulses go to the lateral recti so that the
eyes can converge. Thus both the eyes converge.
Pursuit or Reflex Vergence

In this, the impulses originate from the retina of the two eyes (Fig.
1.9). If a pen is held in front of our eyes and moved towards the nose
and if we keep looking at the pen, then the impulses from the two
eyes will make the eyes converge by the pursuit vergence pathway.
From the retina impulses will go via the optic nerve and tract to area
19 of the occipital lobe. This is a bilateral impulse. From here it goes
to the pretectal area where it reaches the convergence area. From
here impulses pass bilaterally to the III and VI nerve nuclei. This is
the supranuclear pathway. Then positive impulses go to the medial
recti and inhibitory impulses to the lateral recti and the eyes converge
while looking at the object.
Fig. 1.8: Voluntary vergence pathway

pathway
NON-OPTIC REFLEX SYSTEM

The non-optic reflex system integrates eye movements and the body
movements. There are basically three systems in this: (i) semicircular
canals, (ii) neck receptors, and (iii) the cerebellum. The characteristics
are shown in Table 1.1.
Fig. 1.9: Pursuit or reflex vergence pathway

LR- Lateral rectus; MR- Medial rectus; LE- Left eye; RE- Right eye; III- III Cranial
nerve nucleus; VI- VI Cranial nerve nucleus; PGC- Pontine gaze center; MLF-
Medial longitudinal fasciculus; UMN Pathway- Upper motor neuron pathway; LMN
Pathway- Lower motor neuron pathway
Semicircular Canals
If a lateral semicircular canal is stimulated, the non-optic reflex system
starts to work. If the head is rotated to the left (Fig. 1.10), the lateral
semicircular canal is stimulated. If we tilt our head to the left, the
eyes should generally keep looking straight ahead (the ultimate aim
of the whole process). For the eyes to look straight ahead when we
Fig. 1.10: Non-optic reflex system pathway

LR- Lateral rectus; MR- Medial rectus; LE- Left eye; RE- Right eye; III- III Cranial
nerve nucleus; VI- VI Cranial nerve nucleus; PGC- Pontine gaze center; MLF-
Medial longitudinal fasciculus; VN- Vestibular nucleus; UMN Pathway- Upper
motor neuron pathway; LMN Pathway- Lower motor neuron pathway
have tilted our head to the left the eyes will move to the right. Try
this on yourself by tilting your head to the left. You will note your
eyes move to the right so that you keep on looking straight ahead.
When the semicircular canal is stimulated, impulse goes to the same
side (in this case left side) vestibular nucleus. From the left vestibular
nucleus, impulses go to the opposite side pontine gaze center which
in turn send impulses to the right VI nerve nuclei and left III nerve
nucleus. This is the supranuclear pathway. Then the infranuclear
pathway takes over to the right lateral rectus and left medial rectus
and the eyes turn towards the right. This constitutes the vestibular
influence on eye movements.
Neck Receptors
Contributory information also comes from the proprioceptive organs
of the neck muscles via the spinovestibular tract.
Cerebellum
The role of the cerebellum is not very clear. There is a prominent
flocculo-oculomotor tract, which is the only direct cerebellar
connection with the eye nerve nuclei. This pathway connects with the
opposite III nerve nuclei and the same side VI nerve nuclei (exactly
opposite the semicircular canal connection, which connects with the
same side III nerve and opposite side VI nerve nuclei). Thus, the eyes
tend to move in the opposite direction. This pathway may help explain
the reason why nystagmus in cerebellar disease is in the opposite
direction to that occurring in vestibular disease.
POSITION MAINTENANCE SYSTEM

The function of the position maintenance system is to maintain an
object of interest on the fovea or to maintain a specific gaze position.
It is the most complex of eye movements and works efficiently only
when the person is alert. It becomes seriously disturbed when the
persons level of consciousness is depressed. The micromovement
systems use the same substrates as its macrocounterparts, but the
details of the pathways are not yet known.
The micro eye movements are known as microsaccades or flicks
and micropursuits or drifts. The microsystem is continuously active
in maintaining the target precisely on the fovea, presumable while
other eye movement systems are active as well. Hence, it is the ultimate
monitor of eye movements, coordinating all the other eye movement
systems and determining the precise position of the eye with respect
to the target as well as to the head and body. Stated simply, when an
object moves more rapidly than the smooth pursuit system can follow
it, a saccadic compensation is made to maintain the eye position relative
to the moving target. The pursuit system has been overcome by the
position maintenance system.
Take an example of your catching a ball. At that time when the
ball is in the air, your saccadic and pursuit systems work so that your
eyes are on the ball. Sometimes, there would be an overshooting of

either of the systems and at that time the micromovements of
microsaccades and micropursuits take over so that you finally catch
the ball.
SUMMARY
Thus, there are basically five supranuclear pathways, which control
eye movements. It is important to know them if one wants to
understand supranuclear lesions.
REFERENCES
1. Sunita Agarwal, Athiya Agarwal, et al. Textbook of Ophthalmology 4th vol;
Jaypee, India 2003.
2. Amar Agarwal. Handbook of Ophthalmology; Slack USA 2005.
Supranuclear
2 Disorders of
Eye Movements
INTRODUCTION
If paralysis of an eye muscle occurs due to a lesion in the muscle,
nerve or the nerve nucleus, all the functions of the muscle are involved.
For example, if an infranuclear lesion occurs in the medial rectus, the
patient will neither be able to adduct the eye nor be able to perform
convergence as the medial rectus is paralyzed. If the lesion was a
supranuclear lesion, then the patient would not be able to perform
convergence but would be able to adduct the eye. The supranuclear
lesions are lesions above the cranial nerve nucleus.1,2
PSEUDO-OPHTHALMOPLEGIA
In supranuclear lesions, only those activities controlled by the
particular region involved are impaired and other movements even
though carried out by the same muscle remain normal. This paralysis
of one type of movement and not of another is called pseudo-
ophthalmoplegia.
CLASSIFICATION
Depending on the supranuclear pathway, we can classify the supranuclear
lesions as:
Saccadic disorders
Pursuit disorders
Vergence disorders
Non-optic reflex system disorders (Flow chart 2.1).
SACCADIC DISORDERS
Saccadic disorders can in turn be divided into two groups (Flow chart 2.1):
Conjugate palsies
Dissociated palsies.
Flow chart 2.1: Supranuclear pathway lesions
In dissociated palsies, there is a misalignment of the eyes as the

conjugate movements become dissociated, whereas in conjugate palsies
both the eyes fail to look in one direction. In dissociated palsies, one
eye fails to move in a particular direction, whereas the other eye
moves in that direction.
CONJUGATE PALSIES
Depending on the site of lesion, conjugate palsies can be grouped and
classified (Flow chart 2.2). The site of lesion could be in the frontal
lobe, basal ganglia, etc. In other words an area subserving the saccadic
pathway if involved would lead to conjugate palsies.
Lesions of the Frontal Cortex
Overactivity
Epileptic seizures arising in the appropriate area of the frontal cortex
cause what are called frontal adversive attacks. In these episodes, the
attack commences with the head and eyes being forcibly deviated
away from the discharging frontal cortex. If the left frontal cortex has
an overactivity due to a discharging focus and area 8 is involved, the
saccadic system overworks and the eyes look to the opposite side
that is to the right (Fig. 2.1).
The side of the body to which the deviation has occurred may
then be involved by focal motor activity and ultimately the attack
may progress to a generalized seizure (Fig. 2.2).
Supranuclear Disorders of Eye Movements 19
Flow chart 2.2: Conjugate palsies
Fig. 2.1: Frontal lobe overactivity: Frontal adversive seizure

nerve nucleus; PGC- Pontine gaze center; MLF- Medial longitudinal fasciculus
Fig. 2.2: Frontal adversive attack

Occ.Lobe- Occipital Lobe; Fron.lobe- Frontal lobe; PGC- Pontine gaze center
Unilateral Underactivity
Damage to the frontal eye field by a vascular lesion may render the
patient unable to look to the opposite side. This deficit is rarely seen
as rapid compensation occurs and the eye movements appear to be
normal within hours. However, residual evidence may be found in
the patient having difficulty in maintaining gaze in that direction or
in the development of some nystagmus caused by this weakness when
attempting to do so. If the patient is subsequently comatose or
anesthetized, the eyes will deviate towards the damaged side of the
cortex, because of the unopposed activity of the intact opposite frontal
lobe.
Fig. 2.3: Frontal lobe underactivity

Occ.Lobe- Occipital Lobe; Fron.Lobe- Frontal lobe; PGC- Pontine gaze center
If the left side of the frontal area is damaged (Fig. 2.3), the intact
area 8 on the right side acts. This in turn pushes the eyes to the left
side, in other words, to the side of the lesion. The left hemisphere
causes a right hemiparesis and the eyes thus look away from the para-
lyzed limbs.
Bilateral Underactivity
Bilateral lesions of the frontomesencephalic pathway cause saccadic
palsy in both directions with preservation of pursuit and other eye
movements. Bidirectional saccadic palsy necessitates utilization of head
movements for refixation. The eyes remain locked on the original
object of regard during a rapid head movement. This is called spasm
of fixation. Bilateral saccadic palsies could be congenital or acquired. If
acquired it could be due to multiple sclerosis, Wilsons disease,

Huntingtons chorea or lipidosis.
The most striking feature of this condition is the head thrusts utilized
to accomplish refixations. The head moves in the direction of the
eccentric new target, as there is a saccadic palsy present. When the
head moves, the intact vestibulo-ocular system (non-optic reflex
system) gets activated and the eyes are driven away from the attemp-
ted direction of gaze. So, the patient closes the eyelids thus reducing
the vestibulo-ocular reflex gain and so reduces the amount of head
thrust required. Head rotation overshoots the intended target,
enabling the deviated eyes to fixate upon the object.
Lesions of the Basal Ganglia
Overactivity
The basal ganglia is predominantly concerned with movements in the
vertical plane. Overactivity in the basal ganglia leads to the oculogyric
crisis. This usually consists of a fixed deviation of the eyes in an
upward direction. During this crisis, the patient is incapacitated and
any attempt to recover control of the eyes results merely in a feeble
jerky displacement from the position of spasmodic displacement. The
head is frequently turned in the same direction as the eyes. This occurs
in postencephalitic parkinsonism, posthead injury state, neurosyphilis
or brain tumors.
Underactivity: Progressive Supranuclear Palsy

In progressive supranuclear palsy there is loss of nerve cells, vascular
degenerations and glial reactions in the basal ganglia and midbrain.
The first manifestation of progressive supranuclear palsy is an inability
to make vertical saccades, particularly downward saccades. At this
point, the patients bang their shins, eat off only the top part of their
plates and complain of being unable to read (they cannot look down!).
As the disease progresses, horizontal fast movements become
involved as well. Eventually all fast eye movements are affected and
the pursuit movements become cogwheel.
Lesions of the Collicular Area
Parinauds Syndrome
There are several manifestations of lesions in the collicular area. The
signs are thought to be caused by pressure and distortion of
underlying structures in the midbrain and not by damage to specific

pathways traversing the colliculi. The general name for the clinical
picture produced is known as Parinauds syndrome. Any combination
of impaired upward gaze, impaired downward gaze, pupillary
abnormalities or loss of accommodation reflex can occur. In general,
loss of upward gaze associated with dilated pupils that are fixed to
light suggests a lesion at the level of the superior colliculus. Loss of
downward gaze, normal pupillary reactions to light and loss of
convergence suggest that the lesion is slightly lower in the area of the
inferior colliculus. It could be due to lesions of the pineal gland,
multiple sclerosis, vascular diseases or Wernickes encephalopathy.
A special type of nystagmus is present called retractory nystagmus.
This is a very rare sign of disease in the collicular area and consists of
an inward and outward movement of both eyes when the patient
attempts to look upwards. Presumably, it is produced by all the
extraocular muscles acting simultaneouslyjerking the globe back into
the orbit or attempted upward gazein an attempt to overcome the
inability to look upwards.
DISSOCIATED PALSIES
In dissociated palsies, one eye moves in one direction whereas the
other eye cannot move in the same direction. Thus, there is a
dissociation in the gaze movements. These canbe:
Internuclear ophthalmoplegia
One and one-half syndrome
Dissociated vertical palsies.
Internuclear Ophthalmoplegia
Introduction
Lesions affecting the pathways by which the various ocular nuclei are
linked together, i.e. lesions of the medial longitudinal fasciculus (MLF)
or medial longitudinal bundle produces internuclear ophthalmoplegia.
The MLF connects the III nerve and the VI nerve nuclei. If a lesion
occurs in this there is prevention of the harmonious coordination of
these nuclei in producing conjugate movements. So, one eye carries
out a voluntary movement of gaze whereas the other eye does not,
thus leading to failure of the conjugate (both eyes moving in the same
direction) movement. This leads to a misalignment of the eyes and
thus to diplopia. This feature differentiates the internuclear palsies
from the other supranuclear lesions.
Etiology
Depending on the lesion being unilateral or bilateral, various causes
of internuclear ophthalmoplegia are present (Flow chart 2.3). The
common causes are vascular lesions or multiple sclerosis.
Classification
Internuclear ophthalmoplegia (INO) are grouped into three types.
They can be type I, type II or type III INO.
Type I-INO In type I INO, the lesion is near the III cranial nerve
nuclei including also the convergence area (Fig. 2.4). Essentially there
is paralysis of both medial recti. The impulses coming from the pontine
gaze center go to the VI nerve and III nerve nuclei. As the connections
to the VI nerve nuclei are not affected no disturbance is present in
lateral rectus movements. The eyes are divergent due to bilateral
involvement of the medial recti and there is loss of convergence. It
occurs in hypertensive brainstem lesions and multiple sclerosis.
Divergence may be complicated by skew deviation of the eyes in
which one eye may be up and out and the other eye looks down and
out. There may be a see saw nystagmus present in which the eyes jerk
up and down alternately (Fig. 2.5).
Type II-INO In this relatively common variety of INO, the MLF is
damaged and the medial recti fail to move synchronously with the
lateral recti (Fig. 2.6) on attempted lateral gaze to either side. Yet
when each eye is tested alone, the medial recti function is evident but
incomplete. Test this by covering the abducting eye and making the
adducting eye follow the finger. In type II-INO convergence is normal
Flow chart 2.3: Etiology of inter nuclear ophthalmoplegia

Fig. 2.4: Site of lesions in internuclear ophthalmoplegia

III- III Cranial nerve nucleus; VI- VI Cranial nerve nucleus; PGC- Pontine gaze
center; MLF- Medial longitudinal fasciculus
Fig. 2.5: Type I internuclear ophthalmoplegia

Fig. 2.6: Type II internuclear ophthalmoplegia
as the convergence area is not affected (Fig. 2.4). This occurs in multiple
sclerosis, pontine glioma or in encephalitis.
Type III-INO The third variety of INO occurs in multiple sclerosis.
In this type of INO (Fig. 2.7), none of the eye abducts completely
while adduction is complete. The relay to the VI cranial nerve nuclei
is affected on both sides (Fig. 2.4). If you test the eye individually by
closing the other eye, the eye would abduct differentiating this from
an infranuclear lesion (VI nerve palsy).
One and One-Half Syndrome

One and one-half syndrome is also known as paralytic pontine
exotropia. In the primary position the eye which is opposite the side
of lesion is exotropic. The eye on the same side of the lesion looks
straight ahead. The lesion is in the pontine paramedian reticular
Fig. 2.7: Type III internuclear ophthalmoplegia
formation (pontine gaze centerPGC) or VI nerve nucleus and

ipsilateral medial longitudinal fasciculus. From figure 2.8 one will
understand that only the VI nerve on the side opposite the side of the
lesion will work. The patient is not able to gaze with either eye
towards the side of the lesion and is not able to adduct the eye on the
side of the lesion (Fig. 2.9). This is why this is called one and one-half
syndrome as one side gaze is absent and on the other side half the
gaze movement only is present.
Dissociated Vertical Palsies

A dissociated palsy may affect the elevators of one eye in a supranuclear
palsy due to a localized lesion close to the nuclei below the point
where the corticofugal pathway for elevation of the eyes bifurcates
into the branches which go to both III nerve nuclei. In this event,
Fig. 2.8: One and one-half syndrome- Site of lesion

III- III Cranial nerve nucleus; VI- VI Cranial nerve nucleus; PGC- Pontine gaze
center; MLF- Medial longitudinal fasciculus
Fig. 2.9: One and one-half syndrome

conjugate vertical movements are dissociated, one eye being incap-

able of elevation in voluntary movements but moving up normally in
Bells phenomenon.
PURSUIT DISORDERS
Overactivity
If there is an overactivity of the parieto-occipital cortex (read vertical
pursuit pathway), seizures originating in the occipital cortex cause
deviation of the eyes to the same side. But in this situation, the
movements will be accompanied by visual hallucinations. These usually
consist of flashing lights and colored blobs. A generalized convulsion
may ensue but focal motor activity, other than the eye movements is
not a feature of a focal seizure arising in the occipital lobe.
Underactivity
Damage to the parieto-occipital cortex leads to the patient not able to
follow a target on the side of the lesion. If the patient has a right
occipital lobe lesion, then the patient would not be able to follow the
targets to the right side. Damage to the parieto-occipital cortex is
often associated with either parietal lobe difficulties, which may make
testing impossible. Similarly, if a homonymous hemianopia coexists
(as it often does if the lesion is a vascular one), the patient may be
unable to follow an object because it keeps vanishing into the
hemianopic field. In these cases, it is essential to keep the object to be
followed just inside the midline, in the intact half of the patients
vision and to move it slowly.
VERGENCE DISORDERS
Paralysis of Convergence
Paralysis of convergence occurs if the lesion is in the pretectal area
affecting the convergence area (read vergence pathways in Chapter 1).
It is characterized by a failure of convergence with crossed diplopia
of the concomitant type. When the eyes view a near object, together
with the absence of any limitation of movement on either eye
inductions or inversions in any part of the field.
Paralysis of Divergence
Paralysis of divergence is characterized by the appearance of a
convergent strabismus with uncrossed diplopia when the eyes view a
distant object.
NON-OPTIC REFLEX SYSTEM DISORDERS
Vestibular System Disorders

The vestibular apparatus (semicircular canals) controls the non-optic
reflex system. Any lesion affecting the semicircular canals, VIII nerve
or the vestibular nuclei will seriously affect the push-pull effect of the
vestibular control of eye movements.
The right-sided vestibular elements, normally push the eyes to
the left. If there is a lesion in the right vestibular apparatus, it will
lead to weakness of the eye movements to the left side. On attempting
gaze to this side, the intact normal vestibular mechanism on the left
side, coupled with the weakness of the damaged right side will force
the eyes to drift back to the midline. To solve this problem, there will
be a quick jerk of the eyes to the left side; i.e. to the side opposite the
side of the lesion. The quick jerk in this case will be occurring to the
left side and the lesion was in the right side. Nystagmus is always
talked in relation to the fast phase of the nystagmus. In this case
(right side vestibular lesion), the slow phase was on the right side
and to correct it a quick jerk or fast phase occurred towards the left
side. Thus, the nystagmus is away from the side of the lesion in a vestibular
disease (Fig. 2.10).
With destruction of the labyrinth by Mnires disease, nystagmus
does not occur, because of central compensation for the absence of
any input. A similar situation exists after acute labyrinthine destruction
when the initial imbalance settles. Similarly, in slowly occurring
damage to the VIII nerve (an acoustic nerve tumor) compensation
often prevents development of nystagmus. When it does occur in this
situation, it reflects brainstem or cerebellar damage from the extension
of the tumor into the cerebellopontine angle.
With central lesions of the vestibular apparatus (multiple sclerosis,
vascular accidents) compensation cannot occur and the nystagmus
and associated symptoms of vestibular damage tend to persist.
Cerebellar Disorder
The exact mechanism of cerebellar nystagmus is not known. When
nystagmus occurs it is opposite that found in a vestibular lesion. In a
right-sided vestibular lesion, the slow phase of the nystagmus is to
the right and the fast phase to the left. This means the nystagmus is to
the left, in other words opposite the side of the lesion. In cerebellar
disease, the fast phase of the nystagmus is on the same side of the
lesion. So, if there is a right-sided cerebellar lesion, the fast phase of
Fig. 2.10: Vestibular lesion

VN- Vestibular nucleus
the nystagmus is towards the right side. This could be due to the
flocculo-oculomotor pathway, which works in the reverse of the
vestibular pathway. The left vestibular pathway pushes the eyes to
the right whereas the left flocculo-oculomotor pathway from the left
cerebellum pushes the eyes to the left.
REFERENCES
Jaypee, India 2003.
3 Nystagmus
DEFINITION
Nystagmus is a rhythmic to and fro oscillation of the eyes.
GENERAL CONSIDERATIONS
The specific neurophysiologic mechanism of nystagmus is not well
understood. Like all eye movements, nystagmus involves all or more
of the five known supranuclear pathways1,2 namely:
Saccadic system pathway
Pursuit system pathway
Vergence system pathway
Non-optic reflex system pathway
Position maintenance system pathway.
In nystagmus, generally the movement in slow phase is in one
direction and the fast phase in the opposite direction. The fast phase
of nystagmus is mediated by the saccadic system under all conditions.
One or more of the other systems mediates the slow phase. It is
important to remember that nystagmus is given its direction based on the
fast phase. This means that if we say a nystagmus is to the right, it
means that the fast phase of the nystagmus is to the right. But actually,
the important point of nystagmus is the slow phase. So actually,
nystagmus should be given its direction depending on the slow phasebut
this is not done. An abnormality in the slow phase is more significant.
But, alas, convention makes us talk only of the fast phase.
The eye position at any given moment results from all the impulses
fed into the III, IV and VI cranial nerve nuclei, from the supranuclear
mechanism, the gaze systems and the gaze centers. Normally the input
is balanced and the eye movements are smoothly coordinated.
Nystagmus develops when the normal balance is interrupted by a
Nystagmus 33
change of stimulus in a gaze system, frequently the vestibular system.

Thus, in jerk nystagmus, a defect in one system results in eye deviation
(slow phase) and repetitive attempts at correction of that deviation
(by fast phases).
In many kinds of nystagmus, the patient has the subjective
experience that the world is moving or oscillopsia. Oscillopsia or
perception of motion of the visual field associated with nystagmus
seems to be present primarily during the slow phase of nystagmus,
during which time the environment appears to move in the direction
of the fast phase. You can demonstrate this for yourself by following
your finger slowly back and forth horizontally in front of you. Notice
that the background appears to move in a direction opposite to your
slow eye movement (if the slow phase of nystagmus is to the left, the
field appears to move to the right).
During saccades (the fast phases of the nystagmus) the background
does not appear to move. Try making saccades by looking rapidly
from one corner of the room to the other. The background will not be
perceived because of an elevated visual threshold. This elevation of
visual threshold actually occurs prior to the start of the saccades.
Some investigators believe that a discharge associated with the
oculomotor activity of the saccade causes an increase of threshold in
the visual afferent system. Other evidence suggests that the elevation
of visual threshold occurs in the retina as a response to the forms and
contour in the visual environment.
Environmental motion perceived during nystagmus occurs
predominantly during the slow phase, but in a direction that happens
to coincide with the direction of the fast phase. Consequently, a patient
with a large amplitude right-beating nystagmus (fast phase to the
right) might state that the room appears to be moving to the right.
During the fast phase of the nystagmus and during all saccades, visual
perception is suppressed.
TERMINOLOGY
Before we proceed further we should understand what certain terms
mean in nystagmus.
Pendular Nystagmus
In this there is an undulatory movement of equal speed and amplitude
in both directions.
Jerky Nystagmus
Jerky nystagmus demonstrates a biphasic rhythm wherein a slow
movement in one direction is followed by a rapid saccadic return to
the original position.
Micronystagmus
Micronystagmus is a term applied to a nystagmus, which is subclinical,
so that it is incapable of being detected with ordinary clinical tests
because of its extremely small amplitude. The diagnosis is apparent
by the fixation pattern, which shows a regular jerky type of nystagmus
with fast and slow phases of extremely small amplitude within the
parafoveal areas so that it may be revealed only by a careful
examination with the visuoscope or direct ophthalmoscope.
Null Zone
The field of gaze in which the intensity of nystagmus is minimal is
termed the null zone.
Neutral Zone
It is that eye position in which a reversal of direction of jerky
nystagmus occurs and in which any of several bidirectional waveforms,
pendular nystagmus or no nystagmus may be present.
Alexanders Law
Jerky nystagmus usually increases in amplitude with gaze in the
direction of the fast component. This is called Alexanders law.
GRADES
Nystagmus is divided into three grades.
Grade I Jerky nystagmus is evident only in the direction of the fast
phase, i.e. on conjugate deviation to one side.
Grade II When in addition, it is evident in the primary position.
Grade III When it is evident in all positions of the eyes.
EXAMINATION OF A CASE OF NYSTAGMUS

There are certain points one should check when one is examining a
case of nystagmus. They are:
Nystagmus 35
Is the nystagmus pendular or jerky?

The fast phase of the nystagmus is on which side?
Grade of nystagmus
Symptoms of nystagmus
Is squint present or not and if present, the type of squint?
Is the nystagmus affected by heads position?
Is the nystagmus worse with the eyes open or with them closed?
Is the nystagmus affected by convergence?
How wide are the ocular excursions?
CLASSIFICATION
Nystagmus can be divided into various groups (Flow chart 3.1).
Ocular nystagmus
Vestibular nystagmus
Cerebellar nystagmus
Central nystagmus
Miscellaneous.
OCULAR NYSTAGMUS
Ocular nystagmus is due to a defect or embarrassment of central vision,
which renders fixation difficult or impossible. It can in turn be either
physiological or pathological. The physiological nystagmus can in turn
be either deviational nystagmus or optokinetic nystagmus.
Flow chart 3.1: Types of nystagmus

Deviational Nystagmus
Deviational nystagmus is also called end-point nystagmus. It is a jerky
nystagmoid movement of a physiological type when the fixation of
the axes are deviated beyond the limits of the field of binocular fixation
and an effort is made to keep them there. It would generally happen
if a person looks in the extreme lateral gaze. The fast phase is in the
direction of deviation. It would also occur if a person is tired or if
there is a paresis of a muscle.
Optokinetic Nystagmus
Introduction
If a target moving in one direction is shown to a person, then the eyes
move in the direction of the target and when the target goes out of
the limit of gaze, the eyes rapidly comes back to the center to refixate
a new target. This is optokinetic nystagmus.
Clinical Test
A simple way to perform the optokinetic nystagmus (OKN) test is to
hold a tailors tape in both our hands. One should stand one meter
away from the patient. Keep one hand stationary and with the other
hand move the tape. The patient looks at the tape. As the tape moves
in one direction, the patient follows the movement of the tape by a
slow eye movement (pursuit). Then there is a fast eye movement
(corrective saccade) to bring back the eyes to refixate on the tape.
Kestenbaums Newspaper Method

The same result can be done with a sheet of a large newspaper moved
slowly in front of the eyes in a direction perpendicular to the lines of
the newspaper.
Barries Ruler Test

One can use a ruler about 12 inches long to perform the OKN test.
The ruler is held with its long edge horizontal and with its short edge
vertical and moved to the right and left of the eye.
Optokinetic Nystagmus Drum

The best method to test OKN is to use the OKN drum. This is a
special drum, which rotates. The drum has black and white stripes
painted on it. As the drum rotates the patient fixates on the stripes.
Nystagmus 37
There is a slow phase towards the direction of movement of the drum

and when the stripes go out of the field of view, the eyes have a fast
phase so that they come back to the center and refixate new stripes
once again.
Pathway
Optokinetic nystagmus has two parts: a slow phase (pursuit), and a
fast phase (saccadic). Let us imagine a tape or target moving in front
of the patients eyes from left to right. When the target moves from
left to right the eyes fixate the target and the image reaches the retina.
From here it goes to the optic nerve, optic chiasma, optic tract and
then reaches the right occipital cortex in area 19 (Fig. 3.1). This area
subserves the pursuit movements. It is important to note that the
Fig. 3.1: Slow phase of optokinetic nystagmus (OKN)

LR- Lateral rectus; MR- Medial rectus; LE- Left eye; RE- Right eye; Occ.lobe-
Occipital lobe; Fron.lobe- Frontal lobe; III- III nerve nuclei; VI- VI nerve nuclei;
PGC- Pontine gaze center; MLF- Medial longitudinal fasciculus
occipital areas mediate horizontal pursuit movements to the ipsilateral

side. In other words, the right occipital lobe mediates horizontal
pursuit movements to the right.
From the occipital lobe, impulses go to the same side pontine gaze
center. In this case, the impulses from the right occipital lobe go to
the right pontine gaze center. From here impulses go to the right VI
nerve nucleus and the left III nerve nucleus. Till here is the supranuclear
pathway. From the right VI nerve nucleus and the left III nerve nucleus
impulses go via the infranuclear pathway to the lateral rectus and the
medial rectus. Thus, the patients eyes move in the direction of the
target.
When the moving target goes away from the field of vision the
eyes which were moving slowly to that side have to come back to
their original position. A fast eye movement does this, in other words
a saccade. This is the corrective saccade. If a stream of cars are going
in front of our vision, then we keep on following one car and when it
goes out of the field of vision our eyes would come and fixate back to
the car in the center of our field of vision. This would be done by the
corrective saccade. As the impulses from the target moving to the
right reaches the occipital lobe (area 19) and the object is going out of
the field of vision, the occipital lobe sends impulses to the ipsilateral
frontal lobe to perform the corrective saccade. In this case the right
occipital lobe (Fig. 3.2) sends impulses to the right frontal lobe (area
8). This means there has to be a communication between the occipital
lobe and the frontal lobe. From the right occipital lobe impulses pass
to the frontal lobe via the parietal lobe.
From the right frontal lobe, impulses then pass to the left pontine
gaze center which in turn sends impulses to the left VI nerve nucleus
and the right III nerve nucleus. This is the supranuclear pathway.
Then, the infranuclear pathway takes over and impulses got to the
respective lateral and medial recti and the eyes move to the left as a
fast eye movement. This is the corrective saccade.
One can illustrate this with an optokinetic drum, which is a drum
with black and white stripes. The drum is rotated and the eyes fixate
on it. When the stripes go away from the field of vision, the corrective
saccade occurs. This leads to a type of nystagmus known as optoki-
netic nystagmus.
Parietal Lobe Lesion

If the person has a parietal lobe lesion, then there is a problem (Fig.
3.3). When the corrective saccade has to work the impulse would not
Nystagmus 39
Fig. 3.2: Fast phase of optokinetic nystagmus (OKN)

Occipital lobe; III- III nerve nuclei; VI- VI nerve nuclei; PGC- Pontine gaze center;
MLF- Medial longitudinal fasciculus
Fig. 3.3: Parietal lobe lesion

Occipital lobe; Fron.lobe- Frontal lobe; III- III nerve nuclei; VI- VI nerve nuclei;
PGC- Pontine gaze center; MLF- Medial longitudinal fasciculus
pass beyond the parietal lobe. Thus, this would lead to a deficit in the
corrective saccade. So a deep parietal lobe lesion causes loss or decrease
of the fast phase of the OKN, when movement of the drum is towards
the side of the lesion.
Type of OKN Abnormalities

There are four types of OKN abnormalities from type I to type IV
(Table 3.1). The problems in OKN could occur if the lesion is in the
pursuit system starting from the retina, optic nerve to the occipital
lobe or in the saccadic system. There also could be a combination of
both systems involved.
Inverse OKN
An inverse OKN, wherein in horizontal movements the more rapid
excursion occurs in the direction of the moving object, can be seen in
cases of congenital nystagmus of ocular origin or in amblyopic
Table 3.1: Types of optokinetic abnormalities

Features Type I Type II Type III Type IV
Slow phase fast phase Combination complex
abnormality abnormality (I and II)
Pursuit Affected Normal Affected Normal
Saccade Normal Affected Normal Normal
Neuroanatomic Posterior Frontomesen- Extensive Occipital
correlation hemispheric cephalic lesion deep lesion on side
lesions on side on side of OKN hemispheric of OKN
of OKN deviation lesion on side abnormality.
abnormality of SOK Disconnection
abnormality syndrome
possible
involving
splenium of
the corpus
callosum
Frequently Hemianopia Hemiparesis Hemianopia, Hemianopsia
associated Hemiparesis
signs
contralateral
to lesion
Ocular Normal Eyes tonically Same as Same as
deviation deviated in type I type I
direction of
moving targets
Nystagmus 41
nystagmus. The inversion is due to the fact that a pre-existing

nystagmus takes precedence over the optokinetic phenomenon and
may thus augment it or interfere with it.
Pathological Ocular Nystagmus
Amaurotic Nystagmus
Nystagmus of pendular or rarely jerky type may occur in those who
have been blind for a long time. The nystagmus is sometimes constant
and at other times it appears only when the attention is aroused.
Amblyopic Nystagmus
This is due to a defect in central vision in both eyes, which precludes
the normal development of the fixation reflex.
Spasmus Nutans
In this the nystagmus occurs with head nodding. It is also called Dunkel
nystagmus. It generally occurs within the first year of life. The cause
appears to be difficulty in maintaining fixation, which is frequently
associated with inadequate light. There is also insufficient control
due to instability of the motor cortical centers in early life.
Miners Nystagmus
This is an acquired occupational disease of the nervous system with
special manifestations in the ocular motor apparatus, occurring in
workers in coalmines (Fig. 3.4). Basically it is due to lack of
illumination. In the early stages which is the latent stage slight
nystagmus starts. Then in the acute stage trembling of the head and
hands occurs with marked nystagmus and a pathognomic attitude of
the head being thrown back. Then the psychopathic stage starts in
which there are cramps, tremors, headaches and insomnia. The
nystagmus is generally pendular in type in the primary position but
frequently changes to the jerky type on lateral gaze. The treatment of
this condition is to give the patient surface work and improve the
general health.
Latent Nystagmus
In this condition, nystagmus is not normally present when both eyes
are open but is elicited on covering either eye. In the classical case the
nystagmus appears on closing one eye. Bilateral jerky nystagmus is
Fig. 3.4: Miners nystagmus
seen with the fast phase towards the uncovered eye. Another condition
is called manifest latent nystagmus, which occurs in patients with
amblyopia or strabismus who although viewing with both eyes open
are fixing monocularly. Again the fast phase is towards the direction
of the intended viewing eye. The phenomenon of latent nystagmus is
particularly evident when the visual acuity of the two eyes are unequal.
Sometimes if one eye has a very poor vision on covering the better
eye instead of nystagmus, a conjugate deviation of both eyes occurs
towards the side of the closed eye. This is calledthe latent deviation
of Kestenbaum. One is not sure of the reason for latent nystagmus. It
could be due to lack of coordination of the supranuclear centers. It
could also be due to the fact that the nystagmus was latent but kept in
check by convergence so that abolition of the impulse to binocular
convergence allowed it to become manifest.
Nystagmus 43
VESTIBULAR NYSTAGMUS
Vestibular Apparatus
The semicircular canals are three fine tubes arranged in the ear. The
lateral semicircular canal is tilted up 30 degrees. Normally the eyes at
rest are in the primary position (Fig. 3.5). Impulses go from each
semicircular canal to the respective vestibular nuclei. From here, the
impulse goes to the opposite pontine gaze center, which in turn
connects to the same side VI nerve nucleus and opposite side III nerve
nucleus. The impulses thus reach the medial and lateral recti and the
eyes are balanced and in the primary position.
Caloric Test
The most easily understood form of vestibular nystagmus is when
performing the caloric test. The patient lies on a couch with the head
Fig. 3.5: Caloric test eyes at rest in primary position

LR- Lateral rectus; MR- Medial rectus; LE- Left eye; RE- Right eye; III- III nerve
nuclei; VI- VI nerve nuclei; VN Vestibular nuclei; PGC- Pontine gaze center;
back at 60 degrees to bring the lateral semicircular canals to the vertical

position. This is because it is easier to produce convection currents in
a vertical column of fluid. The test should not be performed if the
eardrum is perforated. Water is taken at 30 degrees centigrade and
44 degrees centigrade. Normal temperature is 37 degrees centigrade.
One takes the water at 7 degrees centigrade higher and lower than
the normal temperature. The water is run into each ear in turn. A
thermostat is used to keep the temperature steady. 250 ml of water is
allowed to flow over 40 seconds in the standardized test. While the
water is running the patient looks at a point straight ahead. This
produces vertigo and easily observed nystagmus as the canals are
stimulated or inhibited and the eyes are pushed or pulled on either
side. The duration of the nystagmus is timed. The normal duration is
2 minutes and 15 seconds.
When warm water is passed in the left ear (44C), it stimulates the
left semicircular canal. This in turn increases the discharge to the left
vestibular nucleus and thus the right pontine gaze center. This in turn
leads to the eyes deviating to the right (Fig. 3.6). The slow phase of
Fig. 3.6: Caloric test with warm water

Nystagmus 45
nystagmus is thus away from the ear, which is irrigated with warm
water. The eyes try to come back to the original position with a fast
phase towards the left and thus a vestibular nystagmus is created.
When cold water (30C) is passed through the left ear impulses
are inhibited in that side. So the normal right semicircular canal works
and pushes the eyes with a slow phase to the left (Fig. 3.7). The fast
phase then occurs to the right.
Remember nystagmus is always talked of in regard to the fast
phase. A mnemonic to remember the direction of the fast phase in
the caloric test isCOWS (cold opposite, warm same). This means
cold water calorics produce a fast phase to the opposite side and
warm water calorics produce a fast phase to the same side.
Vertical Vestibular Nystagmus

Vertical vestibular nystagmus can be elicited by bilateral caloric
stimulation with the patient recumbent and his or her head flexed 30
degrees above the horizontal plane. Bilateral cold water calorics
produce vertical nystagmus with the fast phase up and the slow phase
Fig. 3.7: Caloric test with cold water

down. Bilateral warm water calorics produce vertical nystagmus with

the fast phase down and the slow phase up. The mnemonic to
remember this is: Cold Slows Things Down. This means that the
cold water produces the slow phase in the downward direction.
Lesions
Vestibular nystagmus could be produced with either a central lesion
or a peripheral lesion. It is very important to differentiate between
the two. The caloric test can differentiate between canal paresis
(peripheral lesion) and directional preponderance (central lesion).
Canal Paresis (Peripheral Lesion)

If the semicircular canal or the VIII nerve are damaged an incomplete
or defective response to both hot or cold water in the affected ear
will be found. In a normal caloric response (Fig. 3.8) hot and cold
water produce a nystagmus for about 2 minutes. If the patient has a
left canal paresis (Fig. 3.9) then neither hot nor cold water will produce
a good nystagmus in the affected ear. The duration of the nystagmus
Fig. 3.8: Normal caloric response

Nystagmus 47
will be less, for example, it could be just 1 minute. In peripheral lesions,

the nystagmus is unidirectional and is horizontal and not vertical. It
is enhanced by removal of ocular fixation and may be positional.
Directional Preponderance (Central Lesion)

The central connections of the vestibular nerve are such that cold
water in one ear has the same effect as hot water in the other. If it is
found that nystagmus cannot be induced to one side it indicates that
the vestibular nucleus of the appropriate side is defective (Fig. 3.10).
This is known as directional preponderance. In left directional
preponderance both the stimuli necessary to produce nystagmus to
the right fail, that means cold in the left ear and hot in the right ear.
This proves that the nerve endings are normal but the brainstem
mechanism for gaze to the left is defective. Central vestibular
nystagmus is bidirectional and is not influenced by removal of ocular
fixation. There is likely to be associated saccadic and pursuit eye
movement disorders.
Fig. 3.9: Left canal paresis. Neither hot nor cold stimuli
produce a full effect in the left ear, i.e. local lesion
Fig. 3.10: Left directional preponderance. Both the two stimuli necessary to produce
nystagmus to the right fail, that means cold in the left ear and hot in the right ear.
This proves that the nerve endings are normal but the brainstem mechanism for
gaze to the left is defective
There are some situations in which both canal paresis and

directional preponderance may be combined. This is often
encountered when an acoustic nerve tumor or other posterior fossa
lesion is displacing the brainstem.
Rotational Nystagmus
Rotation of the head can also produce a rotational nystagmus. If a
lateral semicircular canal is stimulated, the vestibular system starts to
work. If the head is rotated to the left (Fig. 3.11), the left lateral
semicircular canal is stimulated. If we tilt our head to the left, the
eyes should generally keep looking straight ahead (the ultimate aim
of the whole process). For the eyes to look straight ahead when we
have tilted our head to the left the eyes will move to the right. Try
this on yourself by tilting your head to the left. You will note your
eyes move to the right so that you keep on looking straight ahead.
Nystagmus 49
Fig. 3.11: Rotational nystagmus

When the semicircular canal is stimulated, impulse goes to the same

side (in this case left side) vestibular nucleus. From the left vestibular
nucleus, impulses go to the opposite side pontine gaze center which
in turn sends impulses to the right VI nerve nuclei and left III nerve
nucleus. This is the supranuclear pathway. Then the infranuclear
pathway takes over to the right lateral rectus and left medial rectus
and the eyes turn towards the right. This constitutes the vestibular
influence on eye movements.
On cessation of the rotation the right semicircular canal takes over
producing a deviation of the eyes to the left. Thus, the slow phase is
to the left and this is the postrotational nystagmus (Fig. 3.12).
Remember when we use the direction of nystagmus, it is always said
according to its fast phase.
Fig. 3.12: Postrotational nystagmus

Dolls Head Phenomenon

The rotational nystagmus provides a simple method for testing
vestibular responses in an infant or comatose patient. If an infant or
comatose patient is held at arms length and the head tilted slightly
towards the examiner and rotated to the patients left, the infant will
develop a slow tonic deviation to the right and a corrective saccade
to the left. This is the dolls head phenomenon. This is extremely
helpful in confirming the diagnosis of congenital oculomotor apraxia
in infants. In this disorder, the saccadic mechanism is defective. During
rotation of the head, the child will have the slow phase movement of
the eyes opposite the direction of rotation of the head, but the eyes
will not have a fast phase saccadic return.
If you are not able to perform the caloric test in your office, you
could ask the adult patient to spin around several times while you
Nystagmus 51
note the postrotational nystagmus when the spinning is stopped. The

normal patient who is spun to the left will develop postrotational
nystagmus to the right once the spinning is stopped. In other words
the slow phase will be towards the left and the fast phase towards
the right. The environment will appear to move in the direction of
the fast phase.
CEREBELLAR NYSTAGMUS
The exact mechanism of cerebellar nystagmus is not known. When
nystagmus occurs it is opposite that found in a vestibular lesion. In a
right-sided vestibular lesion, the slow phase of the nystagmus is to
the right and the fast phase to the left. This means the nystagmus is to
the left, in other words opposite the side of the lesion. In cerebellar
disease, the fast phase of the nystagmus is on the same side of the
lesion. So, if there is a right sided-cerebellar lesion, the fast phase of
the nystagmus is towards the right side. This could be due to the
flocculo-oculomotor pathway, which works in the reverse of the
vestibular pathway. The left vestibular pathway pushes the eyes to
the right whereas the left flocculo-oculomotor pathway from the left
cerebellum pushes the eyes to the left.
CENTRAL NYSTAGMUS
In central nystagmus, the nystagmus is of the jerky type. It is
occasionally present when the eyes are at rest, but usually develops
only when they are deviated to one or the other direction. The
nystagmus is symmetrical. This means that the movement starts at
the same angle of eccentricity and has approximately the same
excursion whether the gaze is directed to one or the other side.
MISCELLANEOUS
Voluntary Nystagmus
Voluntary nystagmus is habit learned and retained. The movements
are pendular and minute and in a horizontal direction.
Hysterical Nystagmus
Hysterical nystagmus is just like voluntary nystagmus but the
oscillations are quicker.
Idiopathic Congenital Nystagmus

In this there is congenital nystagmus without any known cause. This
includes all forms of nystagmus noted at birth or within the prenatal
period. It is usually horizontal but may be vertical, circular or elliptical.
It may be pendular or jerky. Certain important points about congenital
nystagmus are:
It is binocular and there is similar amplitude in both eyes
There is no oscillopsia and it is abolished in sleep
It is dampened by convergence and increased by a fixation effort.
As it is dampened by convergence the child usually has good near
acuity and can do well in school
It is uniplanar. This is the hallmark of congenital nystagmus. Plane
of the nystagmus, usually horizontal remains unchanged in all
positions of gaze including the vertical gaze. This phenomenon is
seen only in three entitiescongenital nystagmus, peripheral
vestibular nystagmus and periodic alternating nystagmus
One can frequently identify a null zone of gaze in which the
nystagmus is least marked and the visual acuity is the best
The patient may manifest a head turn to keep the eyes in the null
zone or alternatively have a muscle surgery to create the same
effect
High astigmatism is frequently found which can be treated by
contact lenses.
Nystagmus Blockage Syndrome

In this there is reduction of nystagmus or blockage of the nystagmus
in some particular gaze. The nystagmus diminishes or disappears with
the willed act of forced convergence while fixating a distant target.
This should not be confused with the dampening of congenital
nystagmus during convergence on a near target.
SYMPTOMS
The various symptoms of nystagmus are oscillopsia, diplopia, tilting
of the head or head nodding.
TREATMENT
The treatment can be treating the cause, use of prisms or surgery in
which Fadens operation is done. The methods to treat nystagmus
are shown in flow chart 3.2. The treatment can be general treatment
where the cause is treated or specific treatment, which can be medical
Nystagmus 53
Flow chart 3.2: Treatment of nystagmus
or surgical. In medical treatment one can improve the visual acuity by

using prisms base out to simulate fusional convergence. One can use
prisms to eliminate anomalous head postures also. For a head turn to
the left, the neutral zone is in dextroversion and a prism base out
before the right eye and base in before the left eye will shift the eyes
conjugately along with the neutral zone towards the primary position.
One can also use occlusion in which partial occlusion of the sound eye
with a neutral density filter to decrease visual acuity in the fixating
eye to a level below that of the amblyopic eye but not dark enough to
elicit the nystagmus is used. Surgically one can perform Fadens
operation in which the required muscle creating the nystagmus is
sutured to the sclera at the equator.
REFERENCES
Jaypee, India 2003.
4 The Pupil
NORMAL PUPIL
The pupil is an aperture present in the center of the iris and controls
the amount of light entering the eye and reaching the retina. There is
generally only one pupil but in some cases one can have more than
one pupil and this condition is called polycoria.
PUPILLARY PATHWAYS
Introduction
When light is shone in one eye, both the pupils constrict.1,2 Constriction
of the pupil to which light is shone is called direct light reflex and that
of the other pupil is called consensual (indirect) light reflex. If both pupils
are illuminated simultaneously, the response summates. This means
the constriction of each pupil is greater than the constriction noted
when only one pupil is illuminated. Rods and cones initiate the light
reflex.
Pupilloconstrictor Light Reflex Pathway
Afferent
The outer segments of the rods and cones are the receptors for both
the visual pathway and the light reflex. When light is flashed on the
eyes, the pupillary fibers run in the optic nerve. From there they cross
in the optic chiasma and reach the optic tract (Fig. 4.1). From the optic
tract they leave the visual pathway fibers (which continue to the lateral
geniculate body) and reach the pretectal nucleus. This is an ill-defined
collection of small cells anterior to the lateral margin of the superior
colliculus. Internuncial fibers connect each pretectal nucleus with the
The Pupil 55
Fig. 4.1: Pupilloconstrictor light reflex pathway
Edinger-Westphal nucleus of both sides. Half of the postsynaptic fibers

from the pretectal area curve around the periaqueductal gray matter
to terminate in the ipsilateral Edinger-Westphal nucleus, while the
other half cross, mainly via the posterior commissure to the
contralateral Edinger-Westphal nucleus. This connection forms the
basis of the consensual light reflex. Any given pretectal neuron behaves
functionally as though it receives similar inputs from each eye and
projects equally in each Edinger-Westphal nucleus.
Efferent
Preganglionic parasympathetic myelinated fibers now go to the ciliary
ganglion via the third cranial nerve. They leave the III nerve in its
branch to the inferior oblique. After reaching the ciliary ganglion they
synapse there. Then postganglionic myelinated fibers pass through
the short ciliary nerves to innervate the sphincter pupillae. Normally,

postganglionic fibers are non-myelinated and this is the only exception
to this rule. These are the parasympathetic fibers.
Sleep Normally, there is a tonic inhibitory input from the cerebral
cortex to the Edinger-Westphal nucleus and it is a diminution of this
input that results in pupillary constriction during sleep.
Functions of the Light Reflex

Pupillary constriction associated with light reflex protects against
excessive bleaching of the visual pigments by reducing the amount
of light entering the eye.
Light reflex helps in light and dark adaptation. This plays a role in
maximizing visual acuity at different light levels. A large pupil
will allow more light and so can allow greater acuity in dim
illumination. On the other hand, a smaller pupil will limit the aberra-
tions produced by the eyes refractive system and so can also allow
greater acuity. It has been found that at any given light level, the
size of the pupil strikes an optimum balance between these two
factors.
Convergence Near Reflex Pathway

Near reflex occurs on looking at a near object. It consists of two
componentsa convergence reflex and an accommodation reflex. The
convergence reflex comprises convergence of the visual axes of the
eyes with associated constriction of the pupil.
Afferent
The afferent starts from the medial recti. This travels centrally via the
third nerve to the mesencephalic nucleus of the fifth nerve. From
here the impulses travel to the convergence center in the tectal or
pretectal region. Internuncial fibers from the convergence center then
go to the Edinger-Westphal nucleus (Fig. 4.2).
Efferent
The efferent pathway is along the third nerve (similar to that of the
light reflex). From the III nerve efferent fibers of convergence reflex
relay in the accessory ciliary ganglion and from there reach the
sphincter pupillae.
The Pupil 57
Fig. 4.2: Convergence reflex pathway
Accommodation Reflex Pathway

The accommodation reflex consists of increased accommodation and
associated constriction of the pupil.
Afferent
The afferent starts from the retina. Impulses go from the rods and
cones to the optic nerve, optic chiasma and optic tract (Fig. 4.3). Fibers
then pass to the lateral geniculate body, optic radiations and striate
cortex. The impulses reach area 19 of the occipital cortex. Internuncial
fibers then pass from area 19 to the pontine center via the
occipitomesencephalic tract. From the pontine center fibers pass to
the Edinger-Westphal nucleus of both sides.
Fig. 4.3: Accommodation reflex pathway
Efferent
From the Edinger-Westphal nucleus the efferent impulses travel along
the III cranial nerve and relay in the ciliary and accessory ciliary
ganglion. From there the fibers reach the sphincter pupillae.
Pupillary Dilatation Pathway

The pupillary dilatation starts from the posterior hypothalamus (Fig.
4.4). The pathway is the sympathetic pathway unlike the
pupilloconstrictor pathway which is parasympathetic. From the
hypothalamus first-order neurons start which reach the ciliospinal
center of Budge. These fibers descend uncrossed. This center is located
in the intermediolateral cell column of C8, T1 and T2.
The second-order neuron starts from the ciliospinal center of Budge.
These are the preganglionic fibers and they travel via the inferior and
middle cervical ganglion to synapse in the superior cervical ganglion.
During this long course the preganglionic fibers are closely related to
the apical pleura where they may be damaged by bronchial carcinoma.
This is Pancoasts tumor. They can also be damaged during surgery
on the neck.
The Pupil 59
Fig. 4.4: Pupillary dilatation pathway
The third-order neurons are postganglionic neurons which arise

from the superior cervical ganglion. They ascend along the sympathetic
plexus around the internal carotid artery to enter the skull. The fibers
then join the sympathetic plexus around the ophthalmic artery. Then
they go to the nasociliary nerve (branch of ophthalmic division of the
trigeminal nerve) and pass through the ciliary ganglion. They do not
synapse in the ciliary ganglion and reach the dilator pupillae via the
long ciliary nerves.
Darkness Reflex
When a person goes from a lighted environment to darkness, the
pupils dilate. This dilatation has two causes: the first is simply abolition
of light reflex with consequent relaxation of the sphincter pupillae,
and the second is contraction of the dilator pupillae supplied by the
sympathetic nervous system. The pathways involved in dark reflex
are presumably the same as those of light reflex, since the dilatation
at the end of long light stimulation also involves both relaxation of
the sphincter pupillae and contraction of the dilator pupillae.
Psychosensory Reflex
Psychosensory reflex refers to dilatation of the pupil in response to
sensory and psychic stimuli. A large number of differently described
reflexes fall into this category. They are not seen in a newborn baby,
but appear in the first few days of life, developing fully at the age of
six months. Their mechanism is very complex and their pathways
have still not been elucidated. It is believed that the mechanism of
psychosensory reflexes is a cortical one and the pupillary dilatation
results from two componentsa sympathetic discharge to the dilator
pupillae and an inhibition of the parasympathetic discharge to the
sphincter pupillae.
PUPIL CYCLE TIME

A small beam of light focussed at the pupillary margin induces regular,
persistent oscillations of the pupil. These oscillations can be timed
with a stopwatch. The period of the average complete cycle is called
the pupil cycle time. It is prolonged in optic neuritis and in compressive
optic neuropathy.
LESIONS OF THE PUPIL

There are various lesions, which can occur in the pupil (Fig. 4.5)
depending upon the location of the lesion. They are as follows:
Amaurotic Pupil
Amaurotic pupil is a total afferent pupillary defect (Fig. 4.5). A complete
optic nerve or retinal lesion leading to total blindness on the affected
side causes it. The eye has no perception of light. The points in an
amaurotic pupil are:
The pupil neither reacts to direct light stimulation, nor does it
create a consensual light reflex in the opposite eye
When light is shone on the opposite eye, there is a good light
reflex in that eye and a good consensual light reflex in the affected
eye
This shows that the defect is an afferent defect and the efferent
system is normal.
The Pupil 61
Fig. 4.5: Lesions of the pupil
Marcus Gunn Pupil
Swinging Flashlight Test

A Marcus Gunn pupil is a relative afferent pupillary defect. An
incomplete optic nerve lesion or severe retinal disease causes it. It is
best tested by the swinging flash-light test. To perform this test (Fig.
4.6), a bright flashlight is shone onto one pupil and constriction is
noted. Then the flashlight is quickly moved to the contralateral pupil
and the response is noted. This swinging to and fro of the flashlight
is repeated several times while the pupillary response is observed.
Normally, both pupils constrict equally and the pupil to whom light
is transferred remains tightly constricted. In the presence of a relative
afferent pupillary defect in one eye, the affected pupil will dilate when
the flashlight is moved from the normal eye to the abnormal eye.
Fig. 4.6: Swinging flashlight test
This is a paradoxical response. This is called the Marcus Gunn pupil

and is the earliest indicator of optic nerve disease even in the presence
of a normal visual acuity.
Grades
Marcus Gunn pupil can be graded depending on the response to the
swinging flashlight test. During the swinging flashlight test, if the
amount of light information transmitted from one eye is less than
that carried from the fellow eye, the following phenomenon may be
noted when the light is swung from the normal eye to the defective
eye. Thus Marcus Gunn pupils can be graded.
3-4 + Marcus Gunn pupil: There is immediate dilatation of the pupil,
instead of normal initial constriction
1-2 + Marcus Gunn pupil: No change in pupil size, initially, followed
by dilatation of the pupils
Trace Marcus Gunn pupil: Initial constriction, but greater escape to
a larger intermediate size than when the light is swung back to the
normal eye.
The Pupil 63
Lesion
Marcus Gunn pupil sign indicates an asymmetry of conduction and
will not be present in symmetric bilateral lesions of the optic nerve or
retinal disease. A Marcus Gunn pupil will not occur if the lesion is in
the optic chiasma or optic tract as in these areas fibers are present
from the opposite eye also.
Important Points
There is no such thing as bilateral Marcus Gunn pupil. There may
be bilaterally reduced direct response of the pupils to light,
resulting in light-near dissociation, but the Marcus Gunn
phenomenon requires asymmetry of the afferent light transmission.
Opacities of the ocular media (corneal scar, cataracts or vitreous
hemorrhage) will not cause a Marcus Gunn pupillary phenomenon
if a strong enough flashlight is used.
Extensive retinal damage will cause a significant Marcus Gunn
phenomenon.
Wernickes Hemianopic Pupil

Wernickes hemianopic pupil indicates the lesion in the optic tract
(Fig. 4.5). In this condition, light reflex is absent when light is thrown
on the temporal half of the retina of the affected side and nasal half of
the retina of the opposite side. Light reflex is present when the light
is thrown on the nasal half of the affected side and temporal half of
the opposite side. The patient also has homonymous hemianopia as
the lesion is in the optic tract.
Argyll Robertson Pupil
Lesion
A lesion (neurosyphilis) causes it in the region of tectum (Fig. 4.5).
The lesion is in the region of the sylvian aqueduct when the fibers
from the pretectal nucleus go to the Edinger-Westphal nucleus. An
Argyll Robertson pupil occurs when in addition to involvement of
the internuncial neurons, there is a disturbance of the normal inhibitory
pathways from the reticular activating system upon the
parasympathetic Edinger-Westphal subnucleus. The result of this
inhibition is excessive parasympathetic activity and small pupils.
Features
The vision in the affected eye is normal
There is no reaction to light
The near reflex is normal and the pupils react as the convergence
and accommodation reflex fibers are not affected. The fibers that
mediate the pupillary near response lie ventral to the internuncial
neurons that enter the Edinger-Westphal nucleus
The pupils are miotic and irregular
The pupils dilate very poorly with mydriatics.
Light-Near Dissociation
There are many causes of light-near dissociation. They are shown in Figure
4.5. They are:
Argyll Robertson pupil
Bilateral complete afferent pupillary defect as in bilateral optic
atrophy. In these the convergence reflex pathway (Fig. 4.2) is not
affected as it starts from the medial recti, so there is a light-near
dissociation
Lesions in the pretectal area as once again the near reflex are not
affected. This occurs in Parinauds syndrome.
Pseudo-Argyll Robertson Pupil

In this there is third nerve palsy with aberrant regeneration of medial
rectus innervation into the sphincter innervation pathway.
How to Test for a Pupillary Near Response

The near response should be tested in good room light so that the
patients pupils are midsized and the near object is clearly visible.
The patient is given an accommodative target to look at. Watching
for convergence helps the examiner judge how hard the patient is
trying.
Tonic Pupil
Damage to the ciliary ganglion or short ciliary nerves (Fig. 4.5) produces the
tonic pupil. The features are:
Reaction to light is absent and to near reflex very slow and tonic
Accommodative paresis is present
The affected pupil is larger
It is generally unilateral
The Pupil 65
Cholinergic supersensitivity of the denervated muscle occurs. The

normal pupil does not constrict with 0.125 percent pilocarpine
whereas the tonic pupil does.
It could be due to viral infections affecting the ciliary ganglion like
herpes zoster. It could also be due to diabetes or alcoholism.
When a tonic pupil is associated with absent deep tendon reflexes
in the lower extremities the condition is called Adies tonic pupil. The
lesion is due to denervation of the postganglionic supply of the
sphincter pupillae and ciliary muscle of unknown etiology. It generally
occurs in women.
The near response of the pupil generally exceeds the light reaction
in Adies syndrome. The near response is slow and steady and on
looking back into the distance it tends to hold the contraction for a
few seconds. Thus it is tonic. The reasons for this behavior are not
very well understood. The slowness of the tonic pupil might be due
to the diffusion of acetylcholine through the aqueous to the
supersensitive receptors of the iris sphincter. The near reaction is not
spared in Adies syndromeit is restored. Aberrant regeneration of
fibers occurs that were originally destined for the ciliary muscle into
the iris sphincter. So with every effort to focus the eye on a near
object, impulses spill into the sphincter, constricting the pupil.
Accommodative fibers in the short ciliary nerves outnumber sphincter
fibers by 30 to 1. This means that the ciliary muscle will probably
receive appropriate reinnervation, but the odds against the iris
sphincter receiving the right fibers are very high. Thus with random
regeneration of fibers the power of accommodation is likely to
recover, whereas the light reaction will not. At the same time the
sphincter is likely to be served by aberrant accommodative impulses
that constrict the pupil firmly with every near effort.
Hutchinsons Pupil
Hutchinsons pupil occurs in comatose patients with unilaterally
dilated, poorly reactive pupils. It is due to ipsilateral, expanding
intracranial supratentorial mass (tumor or subdural hematoma) that
is causing downward displacement of the hippocampal gyrus and
uncal herniation across the tentorial edge with entrapment of the third
nerve. The pupillomotor fibers travel in the peripheral portion of the
third nerve and are subject to early damage from compression. This
abnormality typically heralds the onset of a III nerve paralysis and is
an internal ophthalmoplegia. The appearance of an internal
ophthalmoplegia in a patient with a suspected or proven supratentorial
mass is an ominous sign and indicates urgent surgical intervention.
Horners Syndrome
Introduction
In Horners syndrome there is a lesion of the sympathetic system.
There can be three typescentral, preganglionic or postganglionic
(Fig. 4.7). The characteristic features are as follows.
Ptosis There is mild to moderate ptosis due to paralysis of the
Mullers muscle which is supplied by the sympathetic system.
Enophthalmos There can be upside down ptosis also and this is due
to weakness of the inferior tarsal muscle. In this there is elevation of
the inferior eyelid. This leads to an apparent enophthalmos.
Miosis There is moderate miosis due to unopposed action of the
sphincter pupillae following paralysis of the dilator pathway. Pupillary
reactions are normal to light and near. When the lights are turned off
the Horners pupil dilates more slowly than the normal pupil because
it lacks the pull of the dilator pupillae. This is called dilatation lag.
Fig. 4.7: Horners syndrome

The Pupil 67
Facial anhydrosis Reduced sweating on the ipsilateral face and neck

occurs. This is characteristic of preganglionic Horners syndrome.
Heterochromia iridis When the sympathetic ocular innervation is
interrupted early in life (congenital Horners syndrome) the pigment
of the iris stroma fails to develop producing heterochromia iridis.
Central Horners Syndrome

This occurs in lesions located between the hypothalamus and the
ciliospinal center of Budge (Fig. 4.7). It is due to brainstem vascular
lesions or demyelinating lesions. The patient will also have brainstem
signs and sudden onset of vertigo.
Preganglionic Horners Syndrome

In this the lesion is located from C8 toT2 and the superior cervical
ganglion. In other words, the second-order neurons are affected. This
can occur in Pancoasts tumor of the lung or surgery in the neck.
There is anhidrosis of the face and neck as the fibers for sweating
come out from the superior cervical ganglion.
Postganglionic Horners Syndrome

The lesion involves the third-order neurons from the superior cervical
ganglion to the dilator pupillae. It can occur in cavernous sinus lesions
or head trauma. There is ipsilateral vascular headache.
Pharmacological Tests for Horners Syndrome

Cocaine test When 4 percent cocaine is instilled in both eyes, the normal
pupil will dilate but the Horners pupil will not (Fig. 4.8). All Horners
pupils, no matter where the defect in the pathway is located, will
dilate poorly to cocaine. Thus, cocaine helps in establishing the
diagnosis of sympathetic denervation and not in localizing the site of
lesion.
Hydroxyamphetamine test When 10 percent drops of this drug are
instilled into both eyes, in a patient with preganglionic lesion both
pupils will dilate whereas in postganglionic lesions, the Horners pupil
will not. It is because, since the hydroxyamphetamine acts by releasing
norepinephrine from the nerve endings at the myoneural junction, so
in postganglionic lesions, the drug will have no effect.
Adrenaline test or phenylephrine test When either adrenaline 1 in 1000
or phenylephrine 10 percent is instilled in both eyes, the Horners
pupil due to postganglionic lesion dilates more than the normal pupil
because of denervation hypersensitivity.
Fig. 4.8: Pharmacological tests to localize Horners syndrome
Readers Syndrome
This applies to painful postganglionic Horners syndrome. Cluster
headaches are present. Raeders paratrigeminal syndrome is a term
that should probably be limited to the occasional middle fossa mass
that produces trigeminal nerve involvement with pain and a
postganglionic Horners syndrome.
PHARMACOLOGY OF THE PUPIL

The iris has a sphincter controlled by the parasympathetic system
(Fig. 4.9) and a dilator controlled by the sympathetic system (Fig.
4.10). In both the systems acetylcholine is the neurotransmitter at the
synapse between the preganglionic and postganglionic neurons. In
the parasympathetic system, acetylcholine is the neuroeffector at the
sphincter. In the sympathetic system the neuroeffector at the dilator
The Pupil 69
Fig. 4.9: Parasympathetic system
Fig. 4.10: Sympathetic system

is norepinephrine. There are alpha- and beta-sympathetic receptors

in the ciliary body. The beta-receptors are inhibitory. Norepinephrine
acts only upon alpha-receptors, which are the only receptors present
in the dilator muscle of the iris. An iris deprived of postganglionic
innervation develops supersensitivity within 24 to 48 hours, i.e. a
physiologic response occurs when lesser amounts of cholinergic and
sympathetic substances are present than those usually required to
cause the same physiologic response.
Parasympathetic drugs can be of three types: (i) the directly acting
drugs like pilocarpine which act directly on the effector and have the
same action as the acetylcholine, (ii) indirect-acting cholinergic agents
that interfere with the hydrolysis and degradation of acetylcholine,
for example, the anticholinesterase inhibitors like eserine and
physostigmine, and (iii) anticholinergic drugs that compete with
acetylcholine for receptors at the effector site of the sphincter muscle,
such as atropine. Atropine blocks the parasympathetic receptors at
the sphincter muscle and prevents the muscle constriction caused by
acetylcholine.
Sometimes, one has to find out whether a fixed dilated pupil (Fig.
4.11) is due to an interruption of parasympathetic innervation by an
anatomic lesion or to pharmacological dilatation of the pupil. The
examiner should instill 1 to 2 percent pilocarpine in the affected eye.
Fig. 4.11: Dilated pupil

The Pupil 71
If the patient has received an anticholinergic drug that dilated the

pupil by blocking the receptor site, the pupil will not constrict. If the
pupillary dilatation is due to interruption of the parasympathetic
pathways proximal to the neuroeffector junction, the pilocarpine will
act directly on the sphincter muscle and will cause pupillary
constriction.
Sympathetic acting drugs can be directly acting or indirectly acting
drugs. The directly acting drugs like phenylephrine or epinephrine
act directly on the alpha-receptors dilating the pupil. The indirectly
acting drugs like cocaine and hydroxyamphetamine (Paredrine) act
in a different way. Cocaine causes pupillary dilatation by blocking
the normal re-uptake of constantly released norepinephrine. This leads
to an accumulation of norepinephrine at the neuroeffector junction.
Hydroxyamphetamine acts directly on the neuroeffector junction and
causes release of norepinephrine from presynaptic vesicles. The
presence of norepinephrine in presynaptic vesicles depends on the
integrity of the third-order neuron. The presence of norepinephrine
in these vesicles is not impaired by interruption of the preganglionic
(second-order) neuron.
PUPIL ABNORMALITIES
Hippus
Hippus is a visible rhythmic but irregular pupillary oscillation,
deliberate in time and 2 mm or more in excursion. It has no localizing
significance. It occurs in:
Normal person
Presence of total third nerve palsy
Hemiplegia
Multiple sclerosis
Meningitis (acute)
Cerebral syphilis
Myasthenia gravis
Epileptics.
Paradoxical Pupillary Reaction

In this either: (i) the pupil dilates with near vision or constricts in
distant vision, or (ii) the pupil dilates on exposure to light or constricts
when the light is withdrawn. It occurs in:
Syphilis
Tumors of quadrigeminal region
Sleeping individuals who have taken barbiturates

Trauma.
Irregularity of the Pupil

Irregularity of the pupil occurs in:
Congenital coloboma of the iris
Operations on the irislike sector iridectomies
Adherent leukoma as one part of the iris is pulled up to the corneal
scar
Peripheral anterior synechiae
Iritis
Glaucoma
Tumors of the iris or ciliary body
Argyll Robertson pupil
Iridocorneal endothelial syndrome
Optic atrophy.
Polycoria
In this there are more than one pupils present. It occurs in:
Congenital
Surgicaldue to a surgical iridectomy
Iridocorneal endothelial syndrome (essential iris atrophy)
Iridoschisis.
REFERENCES
Jaypee, India 2003.
Visual Pathway
5
Athiya Agarwal
INTRODUCTION
The visual pathway starts from the retina and ends in the cortical
areas.1,2 There are basically seven levels through which the visual
impulses pass. They are: (i) retina, (ii) optic nerve, (iii) optic chiasma,
(iv) optic tract, (v) lateral geniculate body, (vi) optic radiation, and
(vii) cortical areas.
Retina
The end organ of the visual pathway is the neural epithelium of the
rods and cones. The first conducting nerve cell or neuron of the first
order is the bipolar cell. From the bipolar cells the impulses travel to
the ganglion cells (Fig. 5.1). From the ganglion cells to the lateral
geniculate body (LGB) is the second-order neuron and from the LGB
to the occipital cortex is the third-order neuron. This is done via the
optic radiations. In the optic nerve head the peripheral fibers from
the retina insert in the periphery of the disk and those from the central
retina insert in the center of the disk (Fig. 5.2).
Optic Nerve
The optic nerve is the second cranial nerve and is about 5 cm in length.
It has basically 4 portions (read Chapter 6 on Anatomy of the Optic
Nerve). They are:
Intraocular portion
Intraorbital portion
Intracanalicular portion
Intracranial portion.
Fig. 5.1: Neurons in the visual pathway
Fig. 5.2: Arrangement of nerve fibers in the disk from the retina. The peripheral
fibers insert in the periphery of the disk while the central fibers insert in the center
of the disk
Visual Pathway 75
Optic Chiasma
Definition
Optic chiasma is a commissure formed by the junction of the optic
nerve. This provides for crossing of the nasal retinal fibers to the
optic tract of the opposite side and for passage of temporal fibers into
the optic tract of the ipsilateral side.
Dimensions
It is a flattened oblong band, some 12 mm in its transverse diameter
and 8 mm from before backwards.
Types
Central chiasma This is present in about 80 percent of cases. It lies
directly (Fig. 5.3) above the sella, so that expanding pituitary tumors
will involve the chiasma first.
Fig. 5.3: Variations of optic chiasma

Prefixed chiasma This is seen in about 10 percent of cases. In these

cases, the chiasma is present more anteriorly over the tuberculum
sellae. In such a situation, the pituitary tumor may involve the optic
tracts first.
Postfixed chiasma This is seen in about 10 percent of cases. In these
cases, the chiasma is located more posteriorly over the dorsum sellae
so that pituitary tumors are apt to damage the optic nerve first.
Anatomy
The optic chiasma lies over the (Fig. 5.4) the diaphragma sellae and is
ensheathed in pia surrounded by cerebrospinal fluid. As it lies over
the diaphragma sellae, presence of a visual field defect in a patient
with a pituitary tumor indicates suprasellar extension. Posteriorly,
the chiasma is continuous with the optic tracts.
Relations
The relations of the optic chiasma (Figs 5.4 and 5.5) are:
Anteriorlyare the anterior cerebral artery and their anterior
communicating branch.
Laterallyis the internal carotid artery, as it passes upwards after
having pierced the roof of the cavernous sinus. It lies on each side in
contact with the chiasma in the angle between the optic nerve and
tract. Laterally too is the anterior perforated substance. The medial
root of the olfactory tract lies laterally.
Fig. 5.4: Anatomy of the optic chiasma

Visual Pathway 77
Posteriorlyis the tuber cinereuma hollow elevation of gray

matter situated between the mamillaria bodies behind and the optic
chiasma in front. Laterally, it is continuous with the gray matter of
the anterior perforated substance. From its inferior aspect the
infundibulum, which is a hollow conical process, passes downwards
and forwards and through a hole in the posterior part of the
diaphragma sellae attaches itself to the posterior lobe of the pituitary
gland. The infundibulum is thus in close contact with the
posteroinferior part of the chiasma, which it joins at an acute angle.
Aboveis the third ventricle, in the floor of which the chiasma
makes a prominence.
Inferioris the hypophysis (pituitary gland) and under the lateral
edge of the chiasma is the cavernous sinus.
Optic Tract
Definition
Each optic tract is a cylindrical band, which runs from the optic chiasma
to the crus cerebri.
Fig. 5.5: Relations of optic chiasma

Course
It runs laterally and backwards from the posterolateral angle of the
chiasma between the tuber cinereum (Fig. 5.5) and the anterior
perforated substance. Becoming more flattened and strap-like it is
united to the upper part of the anterior then lateral surface of the
cerebral peduncle (crus cerebri).
Relations
The course can be divided into three parts:
Anterior part In the first section of its course (Fig. 5.5), the optic
tract lies superficial on the under aspect of the brain. It runs above
the dorsum sellae and crosses the third nerve from medial to lateral.
Above is the posterior part of the anterior perforated substance and
the floor of the third ventricle while medially is the tuber cinereum.
Middle part In the middle region of its course, the optic tract lies
hidden between the uncus and the cerebral peduncle (crus cerebri). It
is here also the flattening commences to conform to the upper aspect
of the uncus. The optic tract here crosses the pyramidal tract, which occupies
the middle segment of the basis pedunculi. Nearby, just dorsal to the substantia
nigra, are the lemnisci carrying sensory fibers. It thus comes about that a
single lesion here can affect vision and also the great motor and sensory roots.
Posterior part In the posterior part of its course, the optic tract lies
in the depths of the hippocampal sulcus. Below and parallel to it runs
the posterior cerebral artery.
Roots
In the posterior part of its course, the optic tract divides into two
rootsthe medial root and the lateral root.
Medial root The medial root passes to the medial geniculate body.
These are not light fibers but commissural auditory fibers between
the two medial geniculate bodies, whose course in the white matter
is via the optic tracts and chiasma. It is called the Guddens
commissure.
Lateral root This spreads over the LGB and for the most part ends
in it.
Terminations
The fibers of the optic tract coming from the ganglion cells of the
retina reach three major destinations. They are:
Lateral geniculate bodyfor relay to the visual cortex
Visual Pathway 79
Each pretectal nucleusas part of the pupilloconstrictor pathway

Superior colliculusfor general reflex responses to light.
Lateral Geniculate Body
Definition
Lateral geniculate bodies (LGBs) are a pair of bodies, which are part
of the thalamus and form an end station for all fibers subserving
vision in the optic tracts.
Shape
Oval or cap-like structure.
Situation
On the posterior aspect of the thalamus.
Development
During the process of development from lower forms of life, there is
a lateral rotation of the LGB as well as changes in its structure. As a
result, in man, the original external surface has become ventral. This
is of importance as regards the disposition of retinal fibers in the
LGB.
Parts
The LGB is an asymmetrical cone, with a rounded apex to its main
bulk or body and an incomplete rim inferiorly. The rim is drawn out
laterally as a peak or spur, which is largely responsible for the surface
elevation known as the LGB. The anterior part of the rim is observed
by the entry of the optic tract (Fig. 5.6). The medial part of the rim is
superior to the medial root and is variably responsible for the surface
elevation, which appears to lead dorsally into the medial geniculate
body. Inferiorly, the nucleus is hollowed; producing a kind of hilum,
which also extends onto the dorsal aspect of the nucleus which here,
has no rim. The hilum may be represented by a superficial cleft or
depression.
Relations
On coronal section It appears like a peaked cap, the peak projecting
laterally.
Fig. 5.6: Anatomy of the lateral geniculate body (LGB)
On horizontal section It is shown to be related anteriorly with the

optic tract which ends therein. Laterally (Fig. 5.7), it is related to the
retrolenticular portion of the internal capsule. Medially it is related
to the medial geniculate body, posteriorly with the hippocampal
convolution and posterolaterally with the inferior horn of the lateral
ventricle.
On sagittal section It is seen that the fibers of the optic tract divide
into two layers: the inferior of these forms the white layer of the
hilum; and the superior forms the dorsal portion of the saddle. Between
these laminae which form the capsule of the LGB are alternating layers
of myelinated fibers and cells which give the body its characteristic
appearance. From the dorsal portion of the LGB pass a mass of fibers
(which form its peduncle) into the area of Wernicke. This is a small
region of myelinated fibers enclosed by the thalamus medially, the
internal capsule laterally and the LGB posteriorly. The main
constituents of the area of Wernicke are the fibers of the optic
radiation. It also contains the vertical temporothalamic fibers of Arnold.
Superior Brachium
The LGB is connected to the superior colliculus by a slender band
called the superior brachium.
Visual Pathway 81
Fig. 5.7: Relations of the lateral geniculate body
Optic Radiations
Definition
Optic radiation or optic radiation of Gratiolet is a fresh relay of fibers
that carry the visual impulses from the LGB to the occipital lobe.
Course
They pass forwards and then laterally through the area of Wernicke
as the optic peduncle, anterior to the lateral ventricle and traversing
the retrolenticular part of the internal capsule behind the sensory
fibers and medial to the auditory tract. The fibers spread out fanwise
to form the medullary optic lamina.
Meyers Loop
The ventral portion of the optic radiation instead of sweeping back
into the occipital lobe plunges forwards into the temporal pole before
passing backwards as an inferior longitudinal fasciculus of Meyer.
This is known as Meyers loop (Fig. 5.8). Interference with this loop
causes a superior homonymous quadrantic hemianopia.
Fig. 5.8: Optic radiations and Meyers loop
Further Course
The optic radiations as they pass back into the white matter of the
cerebral hemisphere lie deep to the middle temporal gyrus, so that
tumors of this portion of the temporal lobe may give rise to visual
defects. The optic radiations end in the occipital lobe in an extensive
area of thin cortex in which is the white stria of Gennari.
Other Fibers
The other fibers in the optic radiations are:
Fibers that pass from the cortex to the LGB and the superior
colliculus
Descending nerve fibers passing to the ocular motor nuclei.
The Visual Cortex
Calcarine Sulcus
In man, the visual projection cortex is situated along the superior and
inferior lips of the calcarine sulcus (Fig. 5.9). This area is usually called
the striate cortex because of the prominent band of geniculocalcarine
fibers termed as striae of Gennari, after its discoverer who discovered
it in 1776. The striate cortex is also referred to as area 17 of Brodman.
The anterior part of the sulcus is called the calcarine fissure and the
posterior part is called the postcalcarine fissure. The striate cortex is
situated on the inferior and superior lips of the postcalcarine fissure
and on the inferior lips of the calcarine fissure.
Visual Pathway 83
Fig. 5.9: Visual cortex
Course of calcarine sulcus The calcarine sulcus is a deep sulcus

extending from near the occipital pole. The parieto-occipital sulcus
joins the calcarine sulcus at an acute angle a little in front of its middle,
dividing it into anterior and posterior portions and forming an Y-
shaped figure.
Cuneus
If the lips of the parieto-occipital sulcus and calcarine sulcus are widely
separated, it will be seen that although on the surface they appear to
be continuous, they are separated from each other by a small buried
vertical cuneate gyrus called cuneus.
Histology of the Visual Cortex

There are six layers of the visual cortex (Fig. 5.10) histologically. From
external to internal they are:
Layer Iplexiform lamina
Layer IIexternal granular lamina
Layer IIIpyramidal lamina
Layer IVinternal granular lamina: this in turn is divided into
Layer IV A, Layer IV B and layer IV C alpha and Layer IV C beta
Layer Vganglionic lamina
Layer VImultiform lamina.
Fig. 5.10: Histological layers of the cortex
Parastriate Cortex
The visual picture from both the eyes unites in the parastriate cortex
called area 18. The lips of the lunate sulcus separate area 17 from area
19. Area 18 is buried within the walls of the sulcus and is in between
area 17 and area 19.
Peristriate Cortex
This is area 19. Most of area 19 lies in the posterior parietal lobe but
inferiorly it forms part of the temporal lobe. In area 19 the object seen
is recognized.
LOCALIZATION IN THE VISUAL PATHWAY
Retina
The nerve fibers converge towards the disk on the temporal side in
the important papillomacular bundle. There is no overlap between
the upper and lower halves of the fibers of the peripheral parts of the
retina. In the retina the line dividing nasal from temporal fibers, in
the sense of those that will cross the chiasma and those that will not,
passes through the center of the fovea. Hence, the temporal macular
Visual Pathway 85
fibers remain on the same side while the nasal ones cross. The upper
temporal retinal fibers are separated from the lower by the macular
fibersan arrangement, which holds throughout the central visual
pathway.
Optic Nerve
In the optic nerve head In the optic nerve head the arrangement of the
nerve fibers is exactly as in the retina.
In the optic nerve just behind the eyeball Here the nerve fibers are
distributed like in the retina (Fig. 5.11). The upper temporal and lower
temporal fibers which are situated on the temporal half of the optic
nerve and are separated from each other by a wedge-shaped area
occupied by the papillomacular bundle. The upper and lower nasal
fibers are situated on the nasal side.
In the optic nerve near the chiasma Here the macular fibers are
centrally placed (Fig. 5.11).
Fig. 5.11: Arrangement of visual fibers in the optic nerve and tract
Optic Chiasma
The Nasal Fibers

The nasal peripheral fibers constitute about three-quarter of all the
fibers and cross over to enter the medial part of the opposite optic
tract in the following manner.
The lower nasal fibers in the optic nerve traverse (Fig. 5.12) the chiasma
low and anteriorly So they are first affected in the tumors of the pituitary
body producing upper temporal quadrantic field defects. These fibers
form convex loops called Wilbrands knee in the terminal part of the
opposite optic nerve therefore ipsilateral blindness due to lesions of
the proximal most part of the optic nerve is associated with
contralateral field defects. They then cross to the opposite tract and
occupy its lower quadrant.
The upper nasal fibers of the optic nerve traverse the chiasma high and
posteriorly Therefore, they are involved first by lesions coming from
above the chiasma, e.g. craniopharyngioma. After crossing they occupy
the upper nasal quadrant of the opposite optic tract. Some of these
fibers make a loop in the ipsilateral optic tract before crossing.
Fig. 5.12: Arrangement of visual fibers in the optic chiasma

UN Upper nasal fibers; LN Lower nasal fibers; UT Upper temporal fibers; LT
Lower temporal fibers; M Macular fibers
Visual Pathway 87
The Temporal Fibers

The temporal fibers from the retina which occupy the temporal half
of the optic nerves, remain uncrossed and run backwards in the lateral
part of the optic chiasma to reach the dorsolateral part of the optic
tract.
The Macular Fibers

The macular fibers, which occupy the central part at the proximal end
of the optic nerve, keep this position in the anterior part of the chiasma.
Then the crossing (nasal) macular fibers get separated from the
uncrossed fibers and pass as a bundle obliquely backwards and
upwards to decussate in the posterior most part of the chiasma, which
is related to the supraoptic recess. Lesions here may produce central
temporal hemianopic scotoma.
Optic Tract
In the chiasma, crossed and uncrossed fibers are intermingled and
when they reach the optic tract they are rearranged to correspond
with their position in the LGB. The macular fibers (Fig. 5.11) occupy
area of the cross-section dorsolaterally. The fibers from the lower
retinal quadrants are lateral and those from the upper are medial.

The fibers from the upper part of the retina go to the medial part of
the LGB and those from below to the lateral part (Fig. 5.13). The
macular area is somewhat cuneiform and is confined to the posterior
two/third of the nucleus, broadening towards the caudal pole.
The neurons of the LGB go to the visual cortex (Fig. 5.1). The
axons of the ganglion cells synapse with the dendrites of the neurons
of the LGB. There is a regular point to point localization of the retina
in the LGB nucleus, which is also carried from the latter to the visual
cortex. The LGB has 6 lamina (1-6). The crossed fibers end in the
laminae 1, 4 and 6 (Fig. 5.14) while the uncrossed fibers end in the
laminae 2, 3 and 5, in such a way that those from the corresponding
parts of the two retinae end in neighboring part of the adjacent laminae.
Therefore, the smallest lesion of the retina results in degeneration of
three laminae of the LGB in which the retinal fibers end. Hence, the
conducting unit in optic nerve fibers is a 3-laminae unit. Since the
optic radiations commence from all the six laminae (6-laminae unit),
so a lesion in the visual cortex results in degeneration of all the 6
laminae of the LGB.
Fig. 5.13: Arrangement of visual fibers in the

lateral geniculate body and optic radiations
Optic Radiation
In the optic radiations (Fig. 5.13), there occurs a temporal rotation of
the fibers. So, the upper retinal fibers occupy the upper part of the
optic radiations and the lower retinal fibers occupy the lower part of
the optic radiations. The macular fibers lie in the central part of the
optic radiations separating the upper retinal fibers from the lower
retinal fibers.
Visual Cortex
The visual cortex is also called the cortical retina, since a true copy of
the retinal image is formed here. It is only in the visual cortex that the
impulses originating from the two retinae meet. There is a point to
point representation of the retina in the visual cortex. The right visual
cortex is concerned with perception of objects situated to the left of
the vertical median line in the visual fields and left visual cortex with
the objects situated to the right half. In other words, the right visual
cortex receives impulses arising from the temporal half of the right
Visual Pathway 89
Fig. 5.14: Arrangment of the axons of ganglion

cells in the lateral geniculate body
retina and nasal half of the left retina. The left visual cortex receives
impulses arising from the temporal half of the left retina and nasal
half of the right retina.
The visual fibers contained in the optic radiations are relayed in
the visual cortex in the following manner (Fig. 5.15). Fibers from the
macular area relay in an extensive area placed posteriorly in the visual
cortex. Fibers from the peripheral retina end anterior to the macular
fibers. Those from the upper retina go above the calcarine sulcus.
BLOOD SUPPLY OF THE VISUAL PATHWAY
Introduction
The visual pathway receives its blood supply from the two arterial
systems, the carotid and the vertebral connected to each other at the
Fig. 5.15: Arrangement of visual fibers in the visual cortex
base of the brain by the circle of Willis. The branches of the carotid
system which contribute to the blood supply of the visual pathway
are ophthalmic artery, small branches of the internal carotic artery, posterior
communicating artery, anterior cerebral artery and middle cerebral artery.
The arteries of the vertebral systems are cortical, central and choroidal
branches from the posterior cerebral artery. Similar to the brain, the visual
pathway is mainly supplied by the pial network of vessels except the
orbital part of the optic nerve, which is also supplied by an axial system
derived from the central retinal artery.
Retina
Choriocapillaries supply the outer four layers of the retinathe
pigment epithelium layer, the layers of rods and cones, external limiting
membrane, and the outer nuclear layer.
Central retinal artery supplies the inner six layersouter plexiform
layer, inner nuclear layer, inner plexiform layer, ganglion cell layer,
nerve fibre layer, and internal limiting membrane.
The outer plexiform layer gets dual blood supply from chorio
capillaries and central retinal artery. The rational vessels are end
arteries.
Optic Nerve
Read Chapter 6 on Anatomy of the Optic Nerve.
Visual Pathway 91
Fig. 5.16: Blood supply of the visual pathway
Optic Chiasma
The vessels may enter the chiasma directly or through the pial plexus.
The main blood supply of the chiasma (Fig. 5.16) is through the anterior
cerebral artery and the internal carotid artery.
The superior aspect of the chiasma is supplied by branches from
the anterior cerebral artery and the anterior communicating artery
The inferior aspect of the chiasma is supplied by branches form
the internal carotid artery and the posterior communicating artery.
A branch from the ophthalmic artery supplies the anteroinferior
margin of the chiasma.
The venous drainage of the chiasma is as follows:
The superior aspect of the chiasma is drained by the superior
chiasmal vein which ends in the anterior cerebral vein
The inferior aspect of the chiasma is drained by the preinfundibular
vein which drains into the basal vein.
Optic Tract
The pial plexus supplying the optic tract receives contributions from:
Posterior communicating artery
Anterior choroidal artery
Branches from the middle cerebral artery.
Though there is no anastomosis, there is considerable

overlapping in the blood supply of the optic tract by the anterior
choroidal artery and the branches of the middle cerebral artery.
Therefore, occlusion of the anterior choroidal artery does not result
in hemianopia.
The venous drainage from the superior aspect of the optic tract
is through the anterior cerebral vein and from the inferior aspect
of the optic tract through the basal vein.

The blood supply of the LGB is as follows:
Posterior cerebral artery supplies the posteromedial aspect of the
LGB and thus nourishes the fibers coming from the superior
homonymous quadrants of the retina
Anterior choroidal artery almost solely nourishes the anterolateral
aspect of the LGB and thus supplies the fibers coming from the
inferior homonymous quadrants of the retina
The region of the hilum, which contains the macular fibers, is
supplied by a rich anastomosis from both the posterior cerebral
and the anterior choroidal arteries.
Venous drainage of the LGB is through the basal vein.
Optic Radiations
The blood supply of the optic radiations is as follows:
Anterior choroidal artery supplies the optic radiations anteriorly
Deep optic artery (lateral striate artery) which is a branch of the
middle cerebral artery supplies the middle part of the optic
radiations
Calcarine branches of the posterior cerebral artery and perforating
branches from the middle cerebral artery supply the posterior part
of the optic radiations as the fibers spread out to reach the visual
cortex.
Venous drainage from the optic radiations is mainly by the basal
vein and in some parts by the middle cerebral vein.
Visual Cortex
Blood supply of the visual cortex is by:
Posterior cerebral artery supplies the visual cortex mainly through
the calcarine artery
Visual Pathway 93
The terminal branches of the middle cerebral artery supply the

anterior end of the calcarine sulcus and the lateral aspect of the
occipital pole. At the posterior pole, there exists a rich anastomosis
between the posterior and middle cerebral artery.
Venous drainage from the medial aspect of the occipital cortex is
by the internal occipital vein, which ends into the great cerebral
vein of Galen and straight sinus. The superolateral aspect of the
cortex drains into the inferior cerebral vein, which ends in the
cavernous sinus.
LESIONS OF THE VISUAL PATHWAY

AND FIELD DEFECTS
Optic Nerve Type Field Defects

Retinal nerve fibers enter the optic disk in a specific manner (Fig.
5.17). So, nerve fiber bundle defects are of three basic types:
Papillomacular Bundle
Macular fibers enter the temporal aspect of the disk. A defect in this bundle
of nerve fibers results (Fig. 5.18) in one of the following:
Central scotomaa defect covering central fixation
Centrocecal scotomaa central scotoma connected to the blind
spot (the cecum)
Paracentral scotomaa defect of some of the papillomacular fibers
lying next to but not involving central fixation.
Fig. 5.17: Arrangement of nerve fibers in the retinal

Fig. 5.18: Papillomacular bundle field defects
Fig. 5.19: Nerve fiber bundle defects
Arcuate Nerve Fiber Bundle

Fibers from the retina temporal to the disk enter the superior and inferior poles
of the disk. A defect in these bundles (Fig. 5.19) may cause any of the following:
Seidel scotomaa defect in the proximal portion of the nerve fiber
bundle causes a comma-shaped extension of the blind spot called
a Seidels scotoma
Visual Pathway 95
Bjerrum, arcuate or scimitar scotomathis arcuate portion of the

field at 15 degrees from fixation is known as Bjerrums area
Isolated scotoma within Bjerrums areathis is due to a defect of
the intermediate portion of the arcuate nerve fiber bundle
Nasal step of Ronnea defect in the distal portion of the arcuate
nerve fiber bundles produces a nasal step of Ronne. Since the
superior and inferior arcuate bundles do not cross the horizontal
raphe of the temporal retina, a nasal step defect respects the
horizontal (180 degrees) meridian.
Nasal Nerve Fiber Bundle Defects

Fibers that enter the nasal aspect of the disk course in a straight
(nonarcuate) fashion. The defect in this bundle results in a wedge-
shaped temporal scotoma arising from the blind spot and does not
necessarily respect the temporal horizontal meridian.
Remember, nerve fiber bundle defects arise from the blind spot and not
from the fixation point (Fig. 5.20). They do not respect the vertical meridian
but respect the nasal horizontal meridian. If a person has a quadrantic
field defect, then check if the field defect originates from the fixation
point or from the blind spot. If it originates from the fixation point it
is a retrochiasmal lesion and if it originates from the blind spot it is an
optic nerve lesion. Other findings to check for an optic nerve lesion is
decreased visual acuity, which generally will not occur in retrochiasmal
lesions.
Fig. 5.20: Quadrantic field defects differentiation between

retrochiasmal lesion and an optic nerve lesion
Optic Chiasma Lesions

The following defects can occur in optic chiasmal lesions (Figs 5.21
and 5.22).
Bitemporal Hemianopia
The nasal retinal fibers including the nasal half of the macula of each
eye cross in the chiasma, to the contralateral optic tract. The temporal
fibers remain uncrossed. Thus, a chiasmal lesion will cause a bitemporal
hemianopia due to interruption of the decussating nasal fibers.
Central Bitemporal Hemianopia

Macular crossing fibers pass in the posterior part of the chiasma and
are related to the supraoptic recess. Lesions here can produce a central
bitemporal hemianopia.
Junctional Scotoma
A central scotoma in one eye with a superotemporal quadrantic defect
in the other eye indicates a lesion at the junction of the optic nerve
Fig. 5.21: Lesions in the optic chiasma

UN Upper nasal fibers; LN Lower nasal fibers; UT Upper temporal fibers;
LT Lower temporal fibers; M Macular fibers
Visual Pathway 97
Fig. 5.22: Field defects in chiasmal lesions
(RE in this case) and the chiasma. The lower nasal fibers cross in the
chiasma and course anteriorly approximately 4 mm in the contralateral
optic nerve. This is Wilbrands knee (Fig. 5.12). Then they turn back
to join uncrossed lower temporal fibers in the optic tract. A lesion
involving the Wilbrands knee creates the junctional scotoma. An
important gem to remember this is the J Lawton Smith super gem. If a
patient comes with poor vision in the right eye, the important eye for
visual field examination is the left eye. There may be an upper
temporal defect with respect for the vertical meridian, due to
involvement of the Wilbrands knee. The lesion is now intracranial at
the junction of the right optic nerve and chiasma. The field defects
constitute a junctional scotoma.
Upper Temporal Quadrantic Defects

The lower nasal fibers travel low and anteriorly (Fig. 5.12) in the
optic chiasma. Thus, pituitary tumors can affect them. Thus, they
produce upper temporal quadrantic defects.
Lower Temporal Quadrantic Defects

The upper nasal fibers travel high and posteriorly. Thus, a lesion from
above the chiasma like a craniopharyngioma can produce a lesion
here. These produce a lower temporal quadrantic defect.
Optic Tract Lesions

All retrochiasmal lesions result in a contralateral homonymous
hemianopia. In the optic tracts and LGB, nerve fibers of corresponding
points do not yet lie adjacent to one another. This leads to incongruous
visual field defects. When we use the term congruous it means
homonymous hemianopic defects that are identical in all attributes
like location, size, shape, depth and slope of margins. Thus in optic
tract lesions, there is an incongruous homonymous hemianopia
(Fig. 5.23).
Lateral Geniculate Body Lesions

A lesion in the lateral geniculate body is extremely rare. Two types of defects
can occur. They are:
Incongruous homonymous hemianopia
Relatively congruous homonymous horizontal sectoranopia
(Fig. 5.23) associated with sectorial optic atrophy. This is due to
vascular infarction of the LGB.
Fig. 5.23: Field defects in optic tract and lateral geniculate body lesions
Visual Pathway 99
Optic Radiations and Visual Cortex Lesions

Various lesions (Fig. 5.24) can occur in the optic radiations and visual
cortex. Depending on the site of lesion, various field defects can occur.
Temporal Lobe Lesions

Inferior fibers course anteriorly from the LGB into the temporal lobe,
forming Meyers loop, approximately 2.5 cm from the anterior tip of
the temporal lobe. They are separated from the superior retinal fibers,
which course directly back in the optic radiations of the parietal lobe.
Anterior temporal lobe lesions (Fig. 5.25) tend to produce
midperipheral and peripheral contralateral homonymous superior
quadrantanopia. This is called a pie in the sky field defect.
Fig. 5.24: Lesions in the optic radiations and visual cortex

Fig. 5.25: Field defect due to temporal lobe lesions. Anterior temporal lobe lesions
of Meyers loop produce incongruous midperipheral and peripheral contralateral
homonymous superior quadrantanopia. This is a pie in the sky field defect. This
case is an example of a right temporal lobe lesion
Parietal Lobe Lesions

The superior fibers cross directly through the parietal lobe to lie
superiorly in the optic radiations. The inferior fibers course through
the temporal lobe (Meyers loop) and lie inferiorly in the optic
radiations. Thus, there is a correction of the 90 degree rotation of the
visual fibers that occurred through the chiasma into the tracts. Parietal
lobe lesions (Fig. 5.26) tend to produce contralateral inferior
homonymous quadrantanopia as they affect the superior fibers first.
Occipital Lobe Lesions

Central homonymous hemianopia In the visual cortex, the macular
representation is located on the tips of the occipital lobes. A lesion
affecting the tip of the occipital lobe tends to produce a central
homonymous hemianopia (Fig. 5.27).
Macular sparing The macular area of the visual cortex is a watershed
area with respect to the blood supply (Fig. 5.16).
Terminal branches of the posterior cerebral and middle cerebral
arteries supply the macular visual cortex. Only the posterior cerebral
artery supplies the visual cortex subserving midperipheral and
peripheral fields. A more proximal (not terminal) vessel supplies the
area. Therefore, when there is obstruction of flow through the
posterior cerebral artery, ipsilateral macular visual cortex may be
spared, because of blood supply provided by the terminal branches
of the middle cerebral artery. This may be an explanation of macular
sparing (Fig. 5.27). However, when there is a generalized
hypoperfusion state (e.g. intraoperative hypotension), the first area
Visual Pathway 101
Fig. 5.26: Field defect due to parietal lobe lesions. Parietal lobe lesions affect the
superior fibers first and so produce a contralateral inferior homonymous
quadrantanopia. This is a case of a right parietal lobe lesion
Fig. 5.27: Field defects due to occipital lobe lesions

of the visual cortex to be affected is that supplied by terminal branches,

the macular visual cortex, resulting in a central homonymous
hemianopia. To say the patient has macular sparing at least 5 degrees
of the macular field must be spared in both eyes, on the side of the
hemianopia.
Temporal crescents When we fixate with both eyes and achieve fusion
of the visual information gained by both eyes, there is superimposition
of the corresponding portions of the visual fieldsthe central 60
degrees radius of field in each eye. There remains in each eye, a
temporal crescent of field for which there are no corresponding visual
points in the other eye. This temporal crescent of field, perceived by
a nasal crescent of retina, is represented in the contralateral visual
cortex, in the most anterior portion of the mesial surface of the occipital
lobe along the calcarine fissure. If a patient has a homonymous hemi-
anopia with sparing of the temporal crescent (Fig. 5.27), the patient
has an occipital lobe lesion, since this is the only site where the temporal
crescent of fibers are separated from the other nasal fibers of the
contralateral eye.
Riddoch phenomenon This is a rare visual field sign. Riddoch believed
that patients with severe field loss from occipital lobe involvement
perceive from and movement separately. He postulated that perception
of movement recovers before perception of form and that this
phenomenon was of some prognostic value for recovery of field. This
phenomenon is illustrated in the patient with extensive dense
homonymous hemianopia as a result of an occipital lobe lesion. The
patient cannot see a large stationary object in the blind field but can
see a smaller object, if it is moving.
Altitudinal defect Injury to both occipital poles may result in
altitudinal field defects. When the upper portions of the visual cortex
or posterior radiation are damaged, the resultant field defects are
altitudinal with loss of the entire lower field of vision of both eyes. If
the lower portion of the lobes are damaged, death usually occurs
after intracranial bleeding as a result of laceration of dural sinuses.
REFERENCES
Jaypee, India 2003.
Anatomy of
6 the Optic Nerve
Athiya Agarwal
INTRODUCTION
Optic nerve, or the second cranial nerve is 5 cm in length and has four
portions. 1,2 They are: (i) intracranial, (ii) intracanalicular, (iii)
intraorbital, and (iv) intraocular portion.
Although we speak of the optic nerve, it is very important to realize
that it is really no nerve at all, but essentially a fiber tract joining two
portions of the brain. The evidence for this is uncontrollable. They
are:
It is an outgrowth of the brain
Its fibers possess no neurolemmal cells
It is surrounded by the meninges, unlike any peripheral nerve
Both the primary and secondary neurons are in the retina.
COURSE
Intracranial Portion
The optic nerve ensheathed in pia runs as a flattened band from the
anterolateral angle of the somewhat quadrilateral optic chiasma. It
runs forwards and laterally and slightly downwards to the optic
foramen.
Intracanalicular Portion
At its entry into the optic canal, it receives a covering of arachnoid
mater and since the dura mater is prolonged through the canal as a
periosteum, the nerve is in fact from here onwards surrounded by all
three meninges and also of course, the cerebrospinal fluid. It traverses
the optic canal and enters the orbit.
Intraorbital Portion
As a rounded cord, it now runs forwards and slightly laterally and
downwards in a somewhat sinuous manner to allow for ocular
movements and is continued into the back of the eyeball.
Intraocular Portion
It then enters the eyeball just above and 3 mm medial to the posterior
pole.
RELATIONS
Intracranial Portion
The nerve lies at first above the diaphragma sellae, which covers the
pituitary body. Between the two nerves in front of the chiasma is a
triangular space in which is a variable portion of the pituitary, covered
by the diaphragma sellae. Above the nerve is the anterior perforated
substance, the medial root of the olfactory tract and the anterior
cerebral artery, which crosses superiorly to reach its medial side. The
internal carotid artery is at first below and then lateral.
Intracanalicular Portion
The pia forms a sheath closely adherent to the nerve (Fig. 6.1). The
dura constitutes the periosteal lining to the canal and at its orbital
end splits to become continuous on the one hand with the periorbita
and on the other hand with the dura of the optic nerve.
The ophthalmic artery crosses below the nerve in the dural sheath
to its lateral side. It leaves the dura at or near the anterior end of the
canal. Thus, the internal carotid artery is to some extent tied to the
dural sheath by its ophthalmic branch and it is also indirectly attached
to the optic nerve by the adherence of the sheaths and by branches to
the nerve from the ophthalmic artery.
Medial to the optic nerve is the sphenoidal air sinus or a posterior
ethmoidal sinus, from which it may be separated by a thin plate of
bone only. This provides the explanation of retrobulbar neuritis following a
sinus infection.
Intraorbital Portion
At the optic foramen, the nerve is surrounded by the origin of the
ocular muscles. The superior and medial rectus are closely adherent
to the dural sheath. It is this connection which gives rise to the pain in
Anatomy of the Optic Nerve 105
Fig. 6.1: Sheaths of the optic nerve
extreme movements of the globe, so characteristic of retrobulbar neuritis.

Between the optic nerve and the lateral rectus are the divisions of the
III cranial nerve, nasociliary nerve, sympathetic nerves and the VI
cranial nerve.
The nasociliary nerve, ophthalmic artery and superior ophthalmic
vein cross the nerve superiorly between the nerve and the superior
rectus from its lateral to medial side. The ciliary ganglion is lateral to
the nerve between the nerve and the lateral rectus.
The central retinal artery near the optic foramen, runs forwards in
or outside the dural sheath of the nerve. Then it crosses the
subarachnoid space to enter the nerve on its under and medial aspect
about 12 mm behind the eye.
Intraocular Portion
The intraocular portion passes through the sclera and choroid and
finally appears in the eye as the optic disk (Fig. 6.2). The intraocular
portion of the optic nerve head has an average diameter of 1.5 mm,
Fig. 6.2: Optic nerve head
which expands to approximately 3 mm just behind the sclera, where

the neurons acquire a myelin sheath. The optic nerve head may be
arbitrarily divided into the following four portions from anterior to
posterior. They are:
Surface nerve fiber layer
Prelaminar region
Lamina cribrosa
Retrolaminar region.
Surface Nerve Fiber Layer

Surface nerve fiber layer is essentially composed of axonal bundles,
i.e. nerve fibers of the retina, which converge on the optic disk and
astrocytes. The optic disk is covered by a thin layer of astrocytes, the
internal limiting membrane of Elschnig, which separates it from the
vitreous and is continuous with the internal limiting membrane of the
retina (Fig. 6.2). When the central portion of this membrane is
thickened, it is referred to as the central meniscus of Kuhnt. All the
layers of the retina, apart from the nerve fiber layer, near the optic
Anatomy of the Optic Nerve 107
nerve are separated from it by a partial rim of glial tissue called the
intermediary tissue of Kuhnt.
Prelaminar Region
The predominant structures at this level are neurons and a significantly
increased quantity of astroglial tissue. The border tissue of Jacoby
(a cuff of astrocytes) separates the nerve from the choroid.
Lamina Cribrosa
It is a fibrillar sieve-like structure made up of fenestrated sheets of
scleral connective tissue lined by glial tissue. It bridges the posterior
scleral foramina or the scleral canal. The bundles of optic nerve fibers
leave the eye through these fenestrations. A rim of collagenous
connective tissue with some admixture of glial cells, which intervenes
between the choroid and sclera and optic nerve fibers, is called the
border tissue of Elschnig. The lamina cribrosa gets its rich blood supply
from the circle of Zinn.
Retrolaminar Region
This area is characterized by a decrease in astrocytes and the
acquisition of myelin that is supplied by oligodendrocytes. The
addition of myelin sheath nearly doubles the diameter of the optic
nerve from 1.5 to 3 mm. The axonal bundles are surrounded by
connective tissue septa.
BLOOD SUPPLY OF THE OPTIC NERVE
Intracranial Part
This part of the optic nerve is supplied by the periaxial system of vessels. The
pial plexus is contributed by four sources:
Internal carotid artery
Anterior cerebral artery
Ophthalmic artery
Anterior communicating artery.
Intracanalicular Part
The nerve within the optic canal is supplied by the periaxial system of
vessels. The pial plexus in this part is fed mainly by branches from the
ophthalmic artery.
Intraorbital Part
The intraorbital part is supplied by two systems of vessels.
The periaxial systemsupplying the optic nerve is derived from
the 6 branches of the internal carotid artery, namely ophthalmic
artery, long posterior ciliary artery, short posterior ciliary artery,
lacrimal artery, central retinal artery before it enters the optic nerve
and circle of Zinn.
The axial systemsupplying the axial part of the optic nerve is
derived from the intraneural branches of the central retinal artery,
central collateral arteries which come off from the central retinal
artery before it pierces the nerve and central artery of the optic
nerve.
The capillary network for the optic nerve is derived from both the
systems. Anastomosis between the two systems has also been
demonstrated.
Intraocular Part
This is the optic nerve head, which has 4 portions:
The surface nerve fiber layeris mainly supplied by the capillaries
derived from the retinal arterioles. These anastomose with vessels
of the prelaminar region. Occasionally a ciliary-derived vessel from
the prelaminar region may enlarge to form the cilioretinal artery
The prelaminar regionis supplied by vessels of the ciliary region
The lamina cribrosa regionis also supplied by the ciliary vessels
which are derived from the short posterior ciliary arteries and
arterial circle of Zinn-Haller
The retrolaminar regionis supplied by both the ciliary and retinal
circulation with the former coming from the recurrent pial vessels.
The central retinal artery provides centripetal branches from the
pial plexus and also centrifugal branches.
REFERENCES
Jaypee, India 2003.
7 Oculomotor Nerve
Athiya Agarwal
INTRODUCTION
The oculomotor (third cranial) nerve is entirely motor in function.1,2
It supplies all the extraocular muscles except the lateral rectus and
superior oblique. It also supplies the sphincter pupillae and the ciliary
muscle.
NUCLEUS
The nucleus of the oculomotor nerve lies in the midbrain at the level
of the superior colliculus (Fig. 7.1). The oculomotor nucleus complex
has two motor nuclei.
The main motor nucleus. This is composed of the subnuclei supplying
individual extraocular muscles as follows:
Dorsolateral nucleusipsilateral inferior rectus
Intermedial nucleusipsilateral inferior oblique
Ventromedial nucleusipsilateral medial rectus
Paramedial (scattered) nucleuscontralateral superior rectus
Caudal central nucleusbilateral levator palpebrae superioris.
The accessory motor nucleus (Edinger-Westphal nuclei). It is situated
posterior to the main oculomotor nucleus mass. It consists of a median
and two lateral components. Perhaps the cranial half of the nucleus is
concerned with light reflexes and the caudal half with accommodation.
The median part is fork shaped (nucleus of Perlia) and its role in
convergence is questionable.
Important points to remember is that both the LPS are supplied by one
central caudal nucleus and each SR is supplied by the opposite III nerve
nucleus.
Fig. 7.1: Third nerve nucleus
EXIT FROM THE BRAIN

The nerve starts from the third nerve nucleus (Fig. 7.2) and passes
through the red nucleus. It then passes through the corticospinal
(pyramidal) tract and emerges from the midbrain and passes into the
interpeduncular space (Fig. 7.3). The nerve passes between the
posterior cerebral artery and the superior cerebellar artery to reach
the cavernous sinus.
We should at this stage also understand the relations of the other
cranial nerves when they exit from the brain (Fig. 7.3). The IV cranial
nerve comes out dorsally and passes between the posterior cerebral
and superior cerebellar arteries. The V nerve comes out from the
pons. Between the two V nerves is the pons. Lateral to the V nerve is
the middle cerebellar peduncle. The VI, VII and VIII nerve come out
between the pons and medulla oblongata. The VI nerve comes out at
the level of the pyramid (part of medulla oblongata), the VII nerve
comes out at the level of the olive (part of medulla oblongata) and the
VIII nerve comes out at the level of the inferior cerebellar peduncle
(part of medulla oblongata). The IX, X and XI nerves come out between
the olive and the inferior cerebellar peduncle and the XII nerve comes
out between the olive and the pyramid.
Oculomotor Nerve 111
Fig. 7.2: Pathway of oculomotor nerve
Fig. 7.3: Location of exit of cranial nerves in the brain

CAVERNOUS SINUS
The oculomotor nerve is lateral to the posterior clinoid process and
above the attached margin of the tentorium cerebelli. It now lies lateral
to the pituitary fossa above the cavernous sinus, then piercing the
dura it passes through the roof and comes to lie in the lateral wall of
the cavernous sinus (Fig. 7.4).
COURSE IN SUPERIOR ORBITAL FISSURE

The III cranial nerve now enters the superior orbital fissure (SOF) but
just before it does so it divides into a small superior and a larger
inferior division. At about this point the IV nerve crosses the III nerve
and lies above and then lateral to it.
Definition
Superior orbital fissure or sphenoidal fissure is an irregularly linear
fissure situated in the most posterior part of the orbital cavity between
the posterior part of the lateral wall, roof and medial wall of the
orbit.
Size
2 cm long.
Fig. 7.4: Cavernous sinus

Shape
It is comma shaped or retort shaped (Fig. 7.5).
Limbs
It comprises two limbsa narrow lateral part and a wider medial
part.
Borders
Superiorly, the lesser wing of the sphenoid forms the SOF, inferiorly
and laterally it is formed by the orbital process of the greater wing of
the sphenoid, medially by the body of the sphenoid and the orbital
process of the perpendicular plate of the palatine bone.
Relations
The fissure is obliquely placed and its lower end is continuous
anteriorly with the inferior orbital fissure and posteriorly with the
pterygomaxillary fissure. Its medial end is separated from the optic
foramen by the posterior root of the lesser wing of the sphenoid.
Common Tendinous Ring

This stretches across or lies across the fissure. It divides the fissure
into an upper lateral, middle and lower medial part.
Fig. 7.5: Superior orbital fissure

Contents
Passing above the annulus from medial to lateral is the:
Trochlear nerve
Frontal nerve
Lacrimal nerve
Recurrent meningeal branch of the lacrimal artery
Orbital branch of the middle meningeal artery
The superior ophthalmic vein also passes in this part.
Passing within the annulus or between the two heads of the lateral rectus
is the following structures from above downwards;
Superior division of the III cranial nerve
Nasociliary nerve
Sympathetic root of the ciliary ganglion
Inferior division of the III cranial nerve
VI nerve.
As a rule nothing passes below the annulus, but rarely the inferior
ophthalmic vein passes through it.
COURSE IN THE ORBIT

The superior division inclines medially above the optic nerve and just
behind the nasociliary nerve to supply the SR on its undersurface at
the junction of the middle and posterior thirds and the LPS. The
inferior division immediately divides into three. The branch to the
MR passes under the optic nerve to enter the muscle. The branch to
the IR pierces the muscle on its upper aspect near the junction of the
middle and posterior thirds. The long branch to the IO runs along the
floor of the orbit on the lateral border of the IR or between this muscle
and the LR. It crosses above the posterior border of the IO about its
middle and breaks up into 2 or 3 branches, which enter the upper
surface of the muscle. It is this nerve that gives the short stout branch
to the ciliary ganglion for relay to the sphincter pupillae and the ciliary
muscle.
CILIARY GANGLION
Introduction
Ciliary ganglion is a peripheral parasympathetic ganglion placed in
the course of the oculomotor nerve. It lies near the apex of the orbit
between the optic nerve and the tendon of the lateral rectus muscle.
Size
It measures about 2 mm anteroposteriorly and about 1 mm in
diameter.
Color
Reddish gray.
Shape
Polygonal.
Roots
It receives posteriorly (Fig. 7.6) three so, called roots or rami.
Long or Sensory Root

Long or sensory root comes from the nasociliary nerve and is given
off just after that nerve has entered the orbit. It is a slender nerve
about 5 to 10 mm long and passes along the lateral side of the optic
nerve to reach the upper and posterior part of the ganglion. It contains
sensory fibers from the cornea, iris and ciliary body and possibly
sympathetic fibers to the dilator pupillae.
Fig. 7.6: Ciliary ganglion

Short or Motor Root

Short or motor root comes from the nerve to the inferior oblique a
few mm beyond the point where the nerve arises from the inferior
division of the III nerve. It is much thicker than the sensory root and
is about 1 to 2 mm long. It passes upwards and forwards to enter the
posteroinferior angle of the ganglion. It carries parasympathetic fibers
to the sphincter pupillae and the ciliary muscle. These synapse in the
ganglion.
Sympathetic Root
Sympathetic root comes from the plexus around the internal carotid
artery. It passes through the superior orbital fissure within the annulus
tendinosus inferomedial to the nasociliary nerve. It lies below and
close to the long root with which it may be blended and enters the
posterior border of the ganglion between the other roots. It carries
constrictor fibers to the blood vessels of the eye and dilator fibers to
the pupil.
Branches
Short Ciliary Nerve

The somata of the preganglionic parasympathetic nerve fibers reaching
the ciliary ganglion are in the Edinger-Westphal nucleus. They are of
course myelinated. They end in the ganglion by forming synapses
with the somata and dendrites of the postganglionic neurons. These
axons form the short ciliary nerves. They are unique in the fact that
normally postganglionic nerve fibers are not myelinated but they are
the exception to this rule and are myelinated. The short ciliary nerves
contain small groups of displaced ganglion cells. The short ciliary
nerves are 6 to 10 in number. They are delicate filaments, which come
off in two groups from the anterosuperior and anteroinferior angles
of the ganglion respectively. They run sinuously with the short ciliary
arteries above and below the optic nerves, the lower group being the
larger. As they pass forwards, they connect with each other and with
the long ciliaries. Having given branches to the optic nerve and the
ophthalmic artery they pierce the sclera around the optic nerve. They
run anteriorly between the sclera and the choroid, grooving the sclera,
to the ciliary muscle on the surface of which they form a plexus, which
supplies the iris, ciliary body and the cornea (Fig. 7.7).
Fig. 7.7: Supply of short ciliary nerves
BLOOD SUPPLY
All nerves are supplied with blood vessels, which are essential for
their normal functioning. The arteries supplying a nerve are derived
from adjacent vessels, which most often are of a small size. On reaching
the nerve the nutrient artery breaks up into ascending and descending
branches which anastomose in the epineurium with similar branches
from other nutrient arteries. From such epineural vessels, branches
penetrate the perineurium where further anastomoses occurs and
finally small vessels penetrate into the fasciculi and from there a rich
longitudinally disposed capillary network runs up and down the nerve
in unbroken continuity. This intrafascicular network is reinforced along
its length by contributions from the various nutrient vessels, which
reach the epineurium, but no part of the intrafascicular plexus may be
regarded as being dominated by any one nutrient artery. In the same
way the III, IV and VI cranial nerves get their blood supply.
REFERENCES
Jaypee, India 2003.
Lesions of the
8 Oculomotor Nerve
Athiya Agarwal
ETIOLOGY
The common causes of oculomotor nerve palsy are: neoplasms, trauma,
aneurysms, ischemic lesions and others like ophthalmoplegic
migraine.1,2
GENETICS
Oculomotor nerve palsy could be congenital due to a hereditary cause.
The mode of transmission could be either autosomal dominant or
recessive.
CLINICAL FEATURES
Total third nerve paresis may be central, sparing the pupil or peripheral
with pupillary involvement. If the pupil is spared, the most likely
cause is a vascular lesion. If the pupil is involved, it is most likely due
to an aneurysm. The patient has a large exotropia with hypotropia. A
fixed dilated pupil is seen. On attempted adduction, the eye intorts
as the superior oblique would be normal. Excluding birth trauma, the
congenital form of external ophthalmoplegia has certain featuresit
is generally bilateral and the extraocular muscles can vary in their
degree of involvement. One can also get partial paresis as the III nerve
divides into a superior and an inferior division. If the superior division
of the III nerve is involved, generally other cranial nerves are also
involved. One can get an isolated involvement of the inferior division
of the III cranial nerve.
NUCLEAR THIRD NERVE PARESIS

This is extremely rare and occurs if the lesion involves the III nerve nucleus
(Fig. 8.1). The important points about this lesion are:
Lesions of the Oculomotor Nerve 119
Each superior rectus is innervated by the contralateral third nerve

nucleus. So, if there were nuclear third nerve palsy on one side
then there would be a paresis of the contralateral superior rectus.
Both levator palpebrae superioris are innervated by one subnuclear
structure, the central caudal nucleus. Therefore, nuclear third nerve
palsy leads to bilateral ptosis.
THIRD NERVE FASCICLE SYNDROMES

In these cases the III nerve has already left the nucleus, so the lesions
affect only one side. There are various syndromes, which can occur
depending on the site of lesion (Fig. 8.1). They are due generally to
an ischemic, infiltrative (tumor) or rarely inflammatory lesion.
Nothnagels Syndrome
In this case the lesion is in the area of the superior cerebellar peduncle.
As the lesion involves the superior cerebellar peduncle the patient
has an ipsilateral third nerve paresis with cerebellar ataxia.
Benedicts Syndrome
In this syndrome the lesion is in the area of the red nucleus. This
leads to contralateral hemitremor with ipsilateral third nerve paresis.
Fig. 8.1: Syndromes of the oculomotor nerve

Claudes Syndrome
This syndrome has features of both Nothnagels and Benedicts
syndrome.
Webers Syndrome
In this the lesion is in the area of corticospinal (pyramidal) tract. This
leads to an ipsilateral third nerve paresis with contralateral hemiparesis.
UNCAL HERNIATION SYNDROME

As the third cranial nerve goes towards the cavernous sinus, it rests
on the edge of the tentorium cerebelli. The portion of the brain
overlying the third nerve, at the tentorial edge, is the uncal portion
of the undersurface of the temporal lobe. A supratentorial space-
occupying mass located anywhere in or above this cerebral
hemisphere, may cause a downward displacement and herniation of
the uncus across the tentorial edge, thereby compressing the third
nerve. This leads to a dilated and fixed pupil. (Fig. 8.2). This is called
the Hutchinson pupil and is the first indication that altered consciousness
is due to a space-occupying intracranial lesion.
POSTERIOR COMMUNICATING ARTERY ANEURYSM

As the third cranial nerve moves towards the cavernous sinus, it
travels alongside the posterior communicating artery (Fig. 8.3). If there
is an aneurysm of the posterior communicating artery it can lead to
compression of the third nerve. This leads to an isolated third nerve
paresis with the pupil getting involved.
Fig. 8.2: Hutchinson pupil

Lesions of the Oculomotor Nerve 121
Fig. 8.3: Posterior communicating artery aneurysm
CAVERNOUS SINUS SYNDROME

In the cavernous sinus syndrome, there would be third nerve paresis
with involvement of other nerves like IV, V and VI cranial nerves.
The patients have painful ophthalmoplegia. This could be due to
trauma, neoplasms, aneurysms or inflammations. This syndrome can
lead to aberrant regeneration of the III cranial nerve.
ORBITAL SYNDROME
There can be proptosis as an early sign. The V cranial nerve can also
be involved but this would involve only the ophthalmic division.
PUPIL-SPARING ISOLATED THIRD NERVE PARESIS

The pupillomotor fibers travel in the III nerve in the outer layers and
are therefore closer to the nutrient blood supply enveloping the nerve.
This is the reason why the pupillomotor fibers are spared generally
in ischemic third nerve paresis but are affected in compressive lesions
like tumors. Ocular myasthenia can mimic a pupil-sparing third nerve
palsy, so one can perform the Tensilon test to differentiate the two.
ABERRANT REGENERATION OF OCULOMOTOR NERVE

Aberrant regeneration of the cranial nerve follows damage of the
nerve by trauma or tumor.
Lid gaze dyskinesis
Elevation of the lid on adduction (inverse Duanes sign)
Elevation of the lid on depression (pseudo Von Graffes sign)
Pupil gaze dyskinesis

Constriction on adduction (pseudo Argyll Robertson pupil)
Constriction on depression.
If aberrant regeneration occur spontaneously (primary regene-
ration) without a preceding third nerve palsy usually is caused by a
cavernous sinus tumor or aneurysm.
As a rule aberrant regeneration never occurs in Ischemic III nerve
palsy.
MANAGEMENT
Occlusion
One can occlude the paretic eye for sometime, till the healing occurs
and the III nerve paresis is cured.
Medical Treatment
One can give the patient multivitamin injections and tablets and treat
the cause like diabetes or hypertension.
Surgical Treatment
The surgical management of a complete III nerve paralysis is a difficult
job. At the very best, the surgeon will succeed only in moving the
paretic eye into the primary position without restoring adduction,
elevation or depression to a significant degree. A very good method
to treat this condition is to do a tenotomy of the lateral rectus and the
superior oblique combined with a transposition of the vertical recti
muscles to the insertion of the medial rectus muscle. Even though the
treated eye will continue to be immobile, it will at least be centered
and this operation should be considered especially in patients who
fixate with the paralyzed eye. For the ptosis one should perform a
frontalis muscle sling operation. This can be done as a second step.
If the patient has a partial palsy with slight medial rectus movement
one can perform a maximal recession of the lateral rectus muscle (at
least 12 mm) and resection of the medial rectus (at least 7 mm) with
upward transposition of the tendons in case of an associated
hypotropia. This may restore a small but useful field of vision even
though double vision will persist in up and downward gaze.
REFERENCES
Jaypee, India 2003.
Trochlear Nerve
9 and its Lesions
Athiya Agarwal
INTRODUCTION
The trochlear nerve is the fourth cranial nerve is the thinnest and also
has the longest intracranial course of about 75 mm.1,2 This is the only
cranial nerve that comes out from the dorsal aspect of the brainstem.
It is also the only cranial nerve that crosses completely to the opposite
side. In other words, the trochlear nerve arises from the contralateral
nucleus (Fig. 9.1).
NUCLEUS
The trochlear nerve nucleus is situated in the midbrain at the level of
the inferior colliculus. It is caudal to and continuous with the third
nerve nucleus complex.
COURSE
Exit from the Nucleus

From each nucleus, the nerve fibers run laterally and emerge from
the dorsal aspect of the midbrain at the level of the inferior colliculus.
They pass medially and decussate completely. Thus, the IVth cranial
nerve crosses to the opposite side and thus each superior oblique is
supplied from the contralateral trochlear nucleus.
Exit from the Brain

Once the trochlear nerve exits from the brain from the dorsal side it
turns towards the ventral side and passes between the posterior
cerebral artery and superior cerebellar artery. It then pierces the dura
on the posterior corner of the roof of the cavernous sinus to enter
into it.
Fig. 9.1: Trochlear nerve anatomy
Cavernous Sinus
In the cavernous sinus, the nerve runs forwards in the lateral wall
lying below the oculomotor nerve and above the first division of the
fifth cranial nerve. In the anterior part of the cavernous sinus it rises,
crosses over the third nerve and leaves the sinus to pass through the
lateral part of the superior orbital fissure.
Superior Orbital Fissure

The trochlear nerve enters the orbit through the lateral portion of the
superior orbital fissure. The nerve passes medially above the origin
of the levator palpebrae superioris and ends by supplying the superior
oblique muscle through its orbital surface.
Orbital Course
In the orbit, the trochlear nerve leaves the frontal nerve which is at
first close to it at an acute angle and passes medially and forwards
Trochlear Nerve and its Lesions 125
beneath the periosteum and above the levator palpebrae superioris

and superior rectus. It divides up in a fan-shaped manner into 3 or 4
branches, which supply the superior oblique on its upper surface near
the lateral border. The most anterior branch enters the muscle at the
junction of the posterior and middle thirds and the most posterior
some 8 mm beyond its origin.
LESIONS OF THE TROCHLEAR NERVE

Depending on the level of the lesion, various syndromes can occur
with damage to the trochlear nerve. They are as follows:
Nuclear Fascicular Syndrome

It is difficult to distinguish between nuclear and fascicular lesions
due to the short course of the fascicles within the midbrain (Fig. 9.2).
It could be due to hemorrhage trauma or demyelination. It is seen
with contralateral Horners syndrome, since the sympathetic pathways
descend through the dorsolateral tegmentum of the midbrain adjacent
to the trochlear fascicles.
Fig. 9.2: Lesions of trochlear nerve

Subarachnoid Space Syndrome

As the fourth nerve emerges from the dorsal surface of the brainstem,
it can get injured easily. When bilateral fourth nerve palsies occur,
the site of injury is likely in the anterior medullary velum. Contracoup
forces transmitted to the brainstem by the free tentorial edge may
injure the nerves at this site. Other causes could be tumors like
pinealoma or tentorial meningiomas.
Cavernous Sinus Syndrome

If the lesion is in the cavernous sinus, other cranial nerves in close
association with the fourth cranial nerve also get involved.
Orbital Syndrome
In this other cranial nerves close to the fourth cranial nerve are also
involved. Other orbital signs like proptosis, chemosis and conjunctival
injection are also seen. This could be due to trauma, inflammation or
tumors.
Isolated Fourth Nerve Palsy

Isolated fourth nerve palsy could be due to a congenital cause or it
could be acquired. The features of a fourth nerve palsy are:
Hyperdeviation
The involved eye is higher as a result of the weakness of the superior
oblique muscle. One should perform the Bielschowskys head tilting
test, as when the head is tilted towards the ipsilateral shoulder the
hyperdeviation becomes more obvious.
Ocular Movements
Depression is limited in adduction. Intorsion is also limited.
Diplopia
Homonymous vertical diplopia occurs on looking downwards. Usually
the vision is single as long as the eyes look above the horizontal plane.
The patient especially notices diplopia when coming down the stairs.
Abnormal Head Posture

To avoid diplopia, the head takes an abnormal head posture towards
the action of the superior oblique muscle, i.e. the face is slightly turned
to the opposite side, chin is depressed and the head is tilted towards
the opposite shoulder.
CHECKING FOURTH NERVE FUNCTION IN

THE SETTING OF A THIRD NERVE PARESIS
The problem to check the fourth cranial nerve function, if a patient
also has a third cranial nerve paresis is that the involved eye cannot
be adducted well due to the third cranial nerve involvement. As the
eye cannot be adducted, one cannot test the vertical action (depression)
of the superior oblique muscle.
To solve this problem, first of all we should note a limbal or
conjunctival landmark like a blood vessel or pterygium. Then, ask the
patient to look down. The patient will not be able to look down as
the eye is abducted and not adducted (due to the third nerve
involvement). But the eye will intort as the superior oblique works.
We should then check from the conjunctival landmark if the eye is
intorting. If the conjunctival landmark is moving, the eye is intorting
and that means the fourth nerve is intact.
BIELSCHOWSKYS HEAD TILTING TEST

The Bielschowskys head tilting test can diagnose which muscle is
paralyzed. Let us first of all look at a case of R/L hypertropia in
which the right eye is at a higher position than the left eye (Fig. 9.3).
R/L Hypertropia
If the patient has a R/L hypertropia, then it could mean that the right
eye is hypertropic in which case the depressors are paralyzed like the
RIR or the RSO. It could also mean that the right eye is in the normal
position but the left eye is hypotropic. This could be due to the elevators
of the left eye being paralyzed like the LIO and the LSR. This is the
first step or I step of the test. Out of the extraocular muscles we have
narrowed down the diagnosis to four muscles.
Now, we perform the II step of the test. In this we ask the patient
to perform dextroversion or levoversion. This means we ask the
patient to look to the right and to the left. If we ask the patient to
look to the right, the right eye could be higher than the left eye. If the
right eye is higher on dextroversion, then it could mean that the RIR
is involved or it could mean that the left eye is hypotropic. This would
be due to a LIO paralysis. In levoversion if the right eye is higher it
could be due to a RSO paralysis. Alternately, it could mean that the
Fig. 9.3: Bielschowskys head tilting test for R/L hypertropia
left eye is hypotropic and this would be due to LSR paralysis. Thus,
we have narrowed down the muscles from 4 to 2.
Finally, we perform the III step in which we tilt the patients head
to the right and then to the left. If we tilt the head to the right, the
right eye will intort and the left eye will extort. This is because nervous
impulses will be sent from the semicircular canals to keep the eyes in
a straight position. Now, remember the superiors are intorters. So, if the
right eye intorts, it means the superiors in that eye (RSR and RSO)
work and if the left eye extorts it means the inferiors of that eye (LIO
and LIR) work. When this happens in the right eye the RIR will not be
used at all as it is an extorter and in the right eye extortion is not
taking place. But, in the left eye, extortion will take place and the LIO
and LIR will work. Now, the LIO is paralyzed and so cannot work.
This will make the LIR only work in that eye and as a balance will not
be maintained between these two muscles the left eye will move down
as the LIR is also a depressor. Thus, one can diagnose the case of LIO.
If we ask the patient to tilt the head to the left, the left eye will
intort and the right eye will extort. In the right eye the extorters will
be the RIR and RIO. Now the RIR is paralyzed and so the RIO will
only work and the right eye will move upwards.
Similarly, we can differentiate between the RSO and the LSR in
the III step. If we tilt the head to the right, the right eye will intort
and the muscles that will work are the RSO and RSR. As the RSO is
paralyzed the RSR will only work and the right eye will move
upwards.
If we tilt the head to the left, the left eye will intort and the muscles
that will work are the LSR and LSO. If the LSR is paralyzed the LSO
will work and the left eye will move down.
L/R Hypertropia
If we now work on the same principle and get the muscle involved in
a L/R hypertropia (Fig. 9.4).
If the patient has a L/R hypertropia, then it could mean that the
left eye is hypertropic in which case the depressors are paralyzed like
the LIR or the LSO. It could also mean that the left eye is in the
normal position but the right eye is hypotropic. This could be due to
the elevators of the right eye being paralyzed like the RIO and the
Fig. 9.4: Bielschowskys head tilting test for L/R hypertropia

RSR. This is the first step or I Step of the test. Out of the extraocular
muscles we have narrowed down the diagnosis to 4 muscles.
Now, we perform the II Step of the test. In this we ask the patient
to perform dextroversion or levoversion. This means we ask the
patient to look to the right and to the left. If we ask the patient to
look to the right, the left eye could be higher than the right eye. If the
left eye is higher on dextroversion, then it could mean that the LSO is
involved or it could mean that the right eye is hypotropic. This would
be due to a RSR paralysis. In levoversion if the left eye is higher it
could be due to a LIR paralysis. Alternately, it could mean that the
right eye is hypotropic and this would be due to RIO paralysis. Thus,
we have narrowed down the muscles from 4 to 2.
Finally, we perform the III step in which we tilt the patients head
to the right and then to the left. If we tilt the head to the left, the right
eye will extort and the left eye will intort. This is because nervous
impulses will be sent from the semicircular canals to keep the eyes in
a straight position. Now, remember the superiors are intorters. So, if the
right eye extorts, it means the inferiors in that eye (LIO and LIR)
work and if the left eye intorts it means the superiors of that eye
(RSO and RSR) work. When this happens, in the left eye the LSO and
LSR should work. Now, the LSO is paralyzed and so cannot work.
This will make the LSR only work in that eye and as a balance will not
be maintained between these two muscles the left eye will move up
as the LSR is also an elevator. Thus, one can diagnose the case of LSO.
If we ask the patient to tilt the head to the right, the left eye will
extort and the right eye will intort. In the right eye the intorters will
be the RSR and RSO. Now the RSR is paralyzed and so the RSO will
only work and the right eye will move downwards.
Similarly, we can differentiate between the LIR and the RIO in the
III step. If we tilt the head to the left, the right eye will extort and the
muscles that will work are the RIO and RIR. As the RIO is paralyzed
the RIR will only work and the right eye will move downwards.
If we tilt the head to the right, the left eye will extort and the
muscles that will work are the LIR and LIO. If the LIR is paralyzed
the LIO will work and the left eye will move up.
MANAGEMENT
Occlusion
When double vision is restricted to downward gaze as in fourth nerve
paralysis, one can occlude the lower third of the spectacle lens before
Table 9.1: Surgical treatment of superior oblique

muscle paralysis (from von Noorden et al)
Class of SO Paralysis Surgical treatment
Class 1 Inferior oblique myectomy
Class 2 Superior oblique tuck (8-12 mm); recession of
contralateral inferior rectus as a secondary procedure
Class 3 Hypertropia of < 25 prism diopters; inferior oblique
myectomy. If there is hypertropia of < 25 prism diopters;
inferior oblique myectomy with superior oblique tuck
Class 4 As in class 3 plus recession of ipsilateral superior rectus
or contralateral inferior rectus
Class 5 Superior oblique tuck with recession of ipsilateral
superior rectus or recession of contralateral inferior
rectus
Class 6 As in classes 1-5 but bilateral surgery
Class 7 Explore trochlea
the paretic eye with semiopaque scotch tape. This can be performed if
the medical condition is not suitable for surgery.
Surgery
Depending on the class of SO paralysis the surgical treatment is done
according to von Noorden (Table 9.1).
REFERENCES
Jaypee, India 2003.
Abducent Nerve
10 and its Lesions
Athiya Agarwal
INTRODUCTION
The abducent nerve (sixth cranial nerve) is a small, entirely motor
nerve that supplies the lateral rectus of the eyeball.1,2
NUCLEUS
The abducent nucleus is situated in the lower part of the pons, close
to the midline (Fig. 10.1). The facial nerve lies close to it and crosses it
and turns around the nucleus to emerge from the brain just adjacent
to the abducent nerve. Medial to the abducent nerve nucleus lies the
medial longitudinal fasciculus and the pontine paramedian reticular
formation (PPRF). Lateral to it lies the fifth cranial nerve and the
sympathetic neuron. Just ventral to it lies the pyramidal tract.
EXIT FROM THE BRAIN

The abducent nerve exits from the brain between the pons and the
medulla oblongata at the level of the pyramid. Next to it lies the
facial nerve and then comes the eighth cranial nerve.
COURSE
The abducent nerve runs from the pons towards the middle cranial
fossa (Fig. 10.2). Just beyond its origin the III, IV and V nerve are
above it (Fig. 10.3). The sixth nerve passes inferior to the inferior
petrosal sinus in an anterolateral direction and runs almost vertically
up the back of the petrous temporal near its apex. It is placed and
held here in a groove, which has a very variable appearance. Having
arrived at the sharp upper border of the bone, it bends forwards
practically at a right angle (Fig. 10.2) under the petrosphenoid ligament
Abducent Nerve and its Lesions 133
Fig. 10.1: Nucleus of the abducent nerve and the brainstem syndromes
Fig. 10.2: Course of abducent nerve

Fig. 10.3: Abducent nerve and its relations
called the Grubers ligament to enter the cavernous sinus. It is thus

passing through a canal called the Dorellos canal.
Cavernous Sinus
In the cavernous sinus, the sixth nerve runs almost horizontally
forwards. In the posterior part of the sinus, the nerve winds around
the lateral aspect of the ascending portion of the internal carotid artery
thus making a second bend this time however with a lateral convexity
(Fig. 10.2). Further forwards the sixth nerve lies below and lateral to
the horizontal portion of the internal carotid artery.
Superior Orbital Fissure

The sixth nerve then passes through the superior orbital fissure to
enter the orbit. The nerve passes through the middle portion of the
superior orbital fissure.
Orbit
In the orbit, the abducent nerve runs forwards and enters the ocular
surface of the lateral rectus muscle (Fig. 10.4) just behind its middle
portion before dividing into 3 to 4 branches.
Fig. 10.4: Abducent nerve and other nerves in the orbit

III A Upper division of oculomotor; III B Lower division of oculomotor; IV
Trochlear nerve; VI Abducent nerve; LPS Levator palpebrae superiors; SR
Superior rectus; LR Lateral rectus; IR Inferior rectus; MR Medial rectus; SO
Superior oblique; IO Inferior oblique
CLINICAL FEATURES OF SIXTH NERVE PALSY
Deviation
In the primary position the eyeball is converged due to the unopposed
action of the medial rectus muscle.
Ocular Movements
Abduction is limited due to weakness of the lateral rectus muscle.
Diplopia
Uncrossed horizontal diplopia occurs, which becomes worse towards
the action of the paralyzed muscle.
Head Posture
The face is turned towards the action of the paralyzed muscle to
minimize diplopia.
LESIONS
Various lesions of the abducent nerve in its course (Fig. 10.5) can
produce various syndromes. They are as follows:
The Brainstem Syndrome

A brainstem lesion of the sixth nerve may also affect the fifth, seventh
and eight cranial nerves and also the cerebellum. The sixth nerve
nucleus has also connections via the medial longitudinal fasciculus
with the III nerve nucleus and so a lesion here produces a gaze palsy.
Three syndromes can occur in the brainstem (Fig. 10.1). They are as
follows.
Millard-Gubler Syndrome
In this the lesion is ventral and involves the facial nerve and the
pyramidal tract. Thus, there is a sixth nerve paresis, ipsilateral VII
nerve paresis and contralateral hemiparesis.
Raymonds Syndrome
In this syndrome the lesion involves only the sixth cranial nerve and
the pyramidal tract. Thus, the patient has a sixth nerve paresis and
contralateral hemiparesis.
Fig. 10.5: Lesions of the abducent nerve

Fovilles Syndrome
In this the lesion is dorsally. As the lesion is dorsal the areas affected
are the medial longitudinal fasciculus, the pontine paramedian reticular
formation, the fifth nerve and the sympathetic neurons. Thus, the
patient has horizontal conjugate gaze palsy, ipsilateral V, VI, VII and
VIII nerve palsies with ipsilateral Horners syndrome.
Subarachnoid Space Syndrome

Elevated intracranial pressure may result in downward displacement
of the brainstem, with stretching of the sixth nerve, which is tethered
at its exit from the pons and in Dorellos canal. This gives rise to
nonlocalizing sixth nerve palsies of raised intracranial pressure. Thirty
percent of patients with pseudotumor cerebri have sixth nerve paresis,
besides papilledema and its visual field changes.
Petrous Apex Syndrome

The sixth nerve passes under the Grubers ligament in the Dorellos
canal. This makes it liable to a lesion.
Gradenigos Syndrome
This is due to a localized inflammation or extradural abscess of the
petrous apex following complicated otitis media. This leads to:
Sixth nerve palsy
Ipsilateral decreased hearing (eighth nerve involvement)
Ipsilateral facial pain in the distribution of the fifth nerve
Ipsilateral facial paralysis.
Pseudo-Gradenigos Syndrome
This is seen in two conditions:
Nasopharyngeal carcinoma This may cause serous otitis media due to
obstruction of the eustachian tube and the carcinoma may subsequently
invade the cavernous sinus causing sixth nerve paresis.
Cerebellopontine angle tumor This may cause sixth nerve paresis with
decreased hearing (VIII nerve), VII nerve palsy, V nerve palsy, ataxia
and papilledema.
Cavernous Sinus Syndrome

In this other nerves in the cavernous sinus also are involved like the
third, fourth and fifth nerves.
Orbital Syndrome
In this proptosis is an early sign and the optic nerve may appear normal
or demonstrate atrophy or edema. The ophthalmic division of the
fifth nerve is involved. The third, fourth and sixth nerves are also
involved. It occurs due to trauma, tumors or inflammations.
Isolated Sixth Nerve Palsy

This can also occur.
DIFFERENTIAL DIAGNOSIS
Thyroid eye disease
Myasthenia gravis
Duanes syndrome type 1
Spasm of the near reflex
Medial wall orbital blow-out fracture with restrictive myopathy
Break in fusion of a congenital esophoria.
MANAGEMENT
Occlusion
One can perform occlusion when double vision is present in lateral
gaze in patients with mild sixth nerve paresis.
Treatment of the Cause

One should find out the cause and treat it.
Surgery
A maximal recession-resection procedure suffices in most instances
of incomplete abducens paralysis to restore a useful field of single
binocular vision and to eliminate the head turn. If there is a complete
paralysis of the lateral rectus muscle, one can perform a transposition
of the superior and inferior rectus muscles to the insertion of the
lateral rectus muscle. This is called Hummelsheims operation (Fig.
10.6). In this half the SR and LR are transposed to the area of the LR.
Recession of the MR is also done. In Jensens operation also (Fig.
10.7), the transposition is done with recession of the medial rectus. In
this operation, the LR is split and so also the SR and IR. Then the split
portions of the SR and IR are sutured to the split portions of the LR.
Fig. 10.6: Hummelsheims operation. 1. Half the SR and IR are transposed to

the area of the LRl; 2.Recession of the MR is also done
Fig. 10.7: Jensens operation. 1. LR is split and so also the SR and IR are split.
Then the split portions of the SR and IR are sutured to the split portions of the LR;
2. Recession of the MR is also done
Botulinum Toxin Injection

Temporary paralysis of an extraocular muscle can be used in
conjunction with the transposition procedures or in isolation. To
determine the state of recovery of the lateral rectus following a sixth
nerve palsy, a tiny dose of Botulinum toxin is injected into the belly
of the overacting medial rectus muscle. This makes the medial rectus
paralyzed and so the horizontal forces on the globe are more balanced
and the esotropia reduced or eliminated.
REFERENCES
1. Sunita Agarwal, Athiya Agarwal, et al. Textbook of Ophthalmology 4th vol.;
Jaypee, India 2003.
Trigeminal Nerve
11
Athiya Agarwal
INTRODUCTION
The trigeminal nerve1,2 is the fifth cranial nerve and is both motor
and sensory. On the sensory root there is a large ganglion called the
trigeminal ganglion.
NUCLEUS
There are two portions to discuss regarding the nuclei: The first is the motor
nucleus and the second is the sensory nucleus. The motor nucleus is
in the upper pons. The sensory nucleus extends in continuity
throughout the whole length of the brainstem and descends into the
upper 2 or 3 segments of the spinal cord. The mesencephalic part is
for propioception, the pontine part is for nice sensations, and the
spinal or medullary nucleus is for nasty sensations.
EXIT FROM THE BRAIN

The trigeminal nerve exits from the brain at the level of the pons.
Lateral to the fifth nerve is the middle cerebellar peduncle. The motor
nerve emerges separately, slightly cranial and medial to its companion.
TRIGEMINAL CAVE
Together they pass below the tentorium cerebelli to the mouth of the
trigeminal cave. This is a tubular prolongation of arachnoid. The
sensory root expands into a large flat crescentic trigeminal ganglion.
The motor root remains separate. The trigeminal ganglion lies in the
trigeminal or Meckels cave. The anterior half of the ganglion gives
off its three sensory divisions: (i) the ophthalmic division (V1), (ii)
maxillary division (V2), and (iii) mandibular vision (V3). The motor
Trigeminal Nerve 141
root of the nerve has no connection with the ganglion but lies on its
deep surface, crossing from the medial to the lateral side, to join the
mandibular divisions of the trigeminal nerve (Fig. 11.1). The
ophthalmic and maxillary divisions pass forwards in the lateral wall
of the cavernous sinus . They are wholly sensory. The mandibular
division passes straight down from the lower part of the ganglion to
the foramen ovale. Here the motor root (Fig. 11.1) joins it.
OPHTHALMIC DIVISION
In the cavernous sinus, the ophthalmic division picks up sympathetic
fibers from the cavernous plexus. These are for the dilator pupillae
muscle. It divides just posterior to the superior orbital fissure into
three branches, which pass through the superior orbital fissure to
enter the orbit (Fig. 11.1).
Fig. 11.1: Ophthalmic division of the trigeminal nerve

1. Lacrimal nerve; 2. Frontal nerve: (A) Supraorbital N (B) Supratrochlear N;
3. Nasociliary nerve (A) Sensory root to the ciliary ganglion; (B) Long diliary
nerve; (C) Posterior ethmoidal nerve; (D) Anterior ethmoidal nerve; (E) Infratrochlear
nerve
Lacrimal Nerve
This is the smallest branch. It passes through the lateral portion of the
superior orbital fissure lateral to the frontal and IV nerve and above
the superior ophthalmic vein. In the orbit, it runs forwards just lateral
to the upper border of the LR to reach the lacrimal gland. It also
supplies the conjunctiva and skin of the lateral part of the upper lid.
Frontal Nerve
This is the largest of the three branches of the ophthalmic division. It
arises in the cavernous sinus just behind the superior orbital fissure
through which it enters the orbit. In the superior orbital fissure, it is
between the lacrimal nerve and the IV cranial nerve. It runs above
the LPS and divides into two branchesa large supraorbital and a
small supratrochlear nerve.
Nasociliary Nerve
It is intermediate in size between the lacrimal and frontal nerve. It
passes through the superior orbital fissure within the annulus tendon
between the divisions of the third cranial nerve. In the orbit, it inclines
medially with the ophthalmic artery above the optic nerve and below
the SR. Thus, the nasociliary nerve, ophthalmic artery and the superior
ophthalmic vein lie between the optic nerve and the SR muscle. The branches
of the nasociliary nerve are:
Sensory root of the ciliary ganglion This is given off just in front of
the superior orbital fissure. It reaches the ciliary ganglion and does
not synapse there. From the ciliary ganglion about 6 short ciliary nerves
are given off which are sensory to the whole eyeball including the
cornea but not the conjunctiva which is supplied by the lacrimal and
supratrochlear nerves.
Long ciliary nerves They are two in number and come off the
nasociliary nerve. They pierce the sclera and pass in the suprachoroidal
space to supply the iris, ciliary muscle and cornea. They also carry
sympathetic fibers to the dilator pupillae muscle.
Posterior ethmoidal nerve This passes through the posterior ethmoidal
foramen.
Anterior ethmoidal nerve This passes through the anterior ethmoi-
dal foramen. This nerve enters the anterior cranial fossa and reaches the tip
of the nose as the external nasal nerve. This is important as if a patient has
herpes zoster and the tip of the nose is affected it means the nasociliary nerve
is involved and that means the eye will definitely get involved. This is called
Hutchinsons rule.
Trigeminal Nerve 143
Infratrochlear nerve This is the terminal branch of the nasociliary

nerve.
MAXILLARY DIVISION
The maxillary division passes through the foramen rotundum and
gives off three posterior superior alveolar nerves (Fig. 11.2), a middle
superior alveolar nerve and an anterior superior alveolar nerve. Then,
it passes through the infraorbital canal and emerges as the infraorbital
nerve. A loop of nerve called the loop of Woodron connects the
posterior and middle superior alveolar nerves. It also gives off a nerve
to supply the lacrimal gland, which travels along the lacrimal nerve.
It also gives off the zygomatotemporal and zygomatofacial nerves.
MANDIBULAR DIVISION
The mandibular division passes through the foramen ovale and gives
off first the meningeal branch, which passes back into the skull through
the foramen spinosum. It then divides into two divisionsthe anterior
and posterior division. Each in turn has some branches (Fig. 11.3).
Thus, the branches of the mandibular division are:
Meningeal branch
Anterior division which in turn has:
1. Temporal branch
2. Masseteric branch
Fig. 11.2: Maxillary division of the trigeminal nerve

Fig. 11.3: Mandibular division of the trigeminal nerve

1. Meningeal branch; 2. Anterior division ; 3. Posterior division
3. Pterygoid branch
4. Buccal nerve.
Posterior division which in turn has:
1. Auriculotemporal nerve
2. Nerve to medial pterygoid
3. Lingual nerve
4. Inferior alveolar nerve.
REFERENCES
Jaypee, India 2003.
Facial Nerve and
12 its Lesions
Athiya Agarwal
INTRODUCTION
The facial nerve1,2 is the seventh cranial nerve and it is both a motor
as well as a sensory nerve.
NUCLEUS
The seventh cranial nerve has three nuclei:
The Main Motor Nucleus

This lies in the lower part of the pons. The part of the nucleus that
supplies the muscles of the upper part of the face receives corticonuclear
fibers from both cerebral hemispheres. The part of the nucleus that
supplies the muscles of the lower part of the face receives corticonuclear
fibers from the opposite cerebral hemisphere only.
Parasympathetic Nuclei
These include the superior salivatory and lacrimatory nuclei. The
former supplies the submandibular and sublingual glands and the
latter the lacrimal gland.
Sensory Nucleus
This is situated in the upper part of the medulla oblongata.
COURSE
The facial nerve exits from the brain (Fig. 7.3) at the level of the junction
between the pons and the medulla. Medial to it lies the VIth nerve
and lateral to it lies the VIIIth nerve. The nerve then passes through
the internal auditory meatus (Fig. 12.1). At its exit from the brain a
cerebellopontine angle tumor can affect it. In such a case the nerves
affected are the Vth, VIth, VIIth and VIIIth. The geniculate ganglion
is located on the first bend of the facial nerve. This is a sensory
ganglion.
A nerve starts from the geniculate ganglion called the greater
superficial petrosal nerve. This joins another nerve called the deep
petrosal nerve, which is a branch from the sympathetic plexus around
the internal carotid artery. These two nerves join to become the Vidian
nerve or the nerve of the pterygoid canal. This then passes through
the Vidian canal or the Pterygoid canal and ends in the pterygopalatine
ganglion. This ganglion is the largest parasympathetic peripheral
ganglion. It serves as a relay station for secretomotor fibers to the
lacrimal gland and to the mucous glands of the nose, palate and
pharynx. From the pterygopalatine ganglion secretomotor fibers go
to the lacrimal gland. These hitchhike on to V2 (maxillary division of
the trigeminal nerve) and then onto the lacrimal nerve (branch of
Fig. 12.1: Course of the facial nerve

Facial Nerve and its Lesions 147
V1ophthalmic division of the trigeminal nerve). Remember that the

lacrimal glands tear production is due to the VIIth nerve and not due to the
Vth nerve.
From the geniculate ganglion the facial nerve curves downwards
and gives off a nerve called the nerve to the stapedius. If this is cut
the patient develops tinnitus. Then another nerve is given off called
the chorda tympani nerve. This supplies taste for the anterior two-
third of the tongue. The IXth (Glossopharyngeal nerve) nerve supplies
the posterior one-third of the tongue. The VIIth nerve also supplies
submandibular and sublingual glands.
The facial nerve then comes out through the stylomastoid foramen
and gives of the posterior auricular nerve. It then goes to supply the
muscles of the face by dividing into two brancheszygomatotemporal
and cervicofacial. The former gives off the temporal and the zygomatic
branches. The latter gives off the cervical and mandibular branches.
The buccal nerve is between these two branches. Thus, these five
nerves supply the muscles of the face and this distribution is like a
claw of a tiger and hence is called pes anserinus. The zygomatic branch
supplies the orbicularis oculi muscle.
LESIONS OF THE FACIAL NERVE

The lesions of the facial nerve are shown in Figure 12.2. They are as
follows:
Supranuclear Lesion
If the lesion is supranuclear the lower half of the face is only involved
and if it is a lower motor neuron lesion the whole half of the face is
involved. This is because the upper half of the face has a bilateral
innervation.
Cerebellopontine Angle Tumor

Just as the VIIth nerve comes out from the brain it can get affected by
the cerebellopontine angle tumor. The patient has:
Total ipsilateral facial weakness (VIIth nerve involvement)
Decreased tearing (lacrimation involved)
Hyperacusis (nerve to stapedius involved)
Decreased taste from the anterior two-third of the tongue (chorda
tympani nerve involved)
Vth, VIth and VIIIth nerve involved with cerebellar dysfunctions.
Fig. 12.2: Lesions of the facial

1. Supranuclear lesion; 2. Cerebellopontine angle tumor; 3. Geniculate ganglionitis
(Ramsay Hunt syndrome); 4. Isolate ipsilateral tear deficiency; 5. Lesion before
nerve to stapedius; 6. Lesion after nerve to stapedius; 7. Lesion after chorda
tympani nerve; 8. Bells palsy isolated total ipsilateral facial palsy; 9. Isolated
partial ipsilateral facial palsy
Geniculate Ganglionitis
Geniculate ganglionitis is known as the Ramsay-Hunt syndrome. The
features are:
Same findings as in cerebellopontine angle tumors except no
associated neurological deficits
May see zoster vesicles on tympanic membrane, external auditory
canal or external ear.
Facial Nerve and its Lesions 149
Isolated Ipsilateral Tear Deficiency

Isolated ipsilateral tear deficiency occurs in nasopharyngeal carcinomas,
which affect the Vidian nerve or the pterygopalatine ganglion.
Lesion before Nerve to Stapedius

Lacrimation is normal. The other findings are:
Hyperacusis (nerve to stapedius involved)
Total ipsilateral facial weakness (VIIth nerve involvement).
Lesion after Nerve to Stapedius

Total ipsilateral facial weakness (VIIth nerve involvement).
Lesion after Chorda Tympani Nerve

Only total ipsilateral facial weakness (VIIth nerve involvement).
Bells Palsy
Only total ipsilateral facial weakness (VIIth nerve involvement).
Isolated Partial Ipsilateral Facial Palsy

In this only certain branches of the VIIth nerve are affected.
SUMMARY
Thus, if we understand the course of the facial nerve, we can diagnose
the level of lesion in the facial nerve.
REFERENCES
Jaypee, India 2003.
Congenital Optic
13 Nerve Anomalies
Priya Narang, Sameer Narang, Amar Agarwal
INTRODUCTION
Congenital optic nerve anomalies are quite a common entity and are
often included in the differential diagnosis of various clinical disorders
as they are often associated with visual field defects central nervous
system (CNS) malformations and other ocular abnormalities. A
thorough knowledge of the embryological development of the optic
nerve entails a better understanding of the development of optic nerve
anomalies.
The optic nerve develops from the optic stalk or optic pedicle. The
closure of the fetal fissure converts the optic stalk into a rounded
cord-like structure. Its cavity communicates on one side with the cavity
of diencephalon and on the other side with the primary optic vesicle.
The nerve fibers then grow from the ganglion cells towards the brain
through the stalk. The optic nerve is then filled with glial tissue and
fibrous septa. The sheaths of the optic nerve develop from the
mesoderm. The medullation of the nerve fibers which begins from
the brain and extends up to the lamina cribrosa is nearly complete at
term. As stated above, any deviation from the normal development
leads to congenital anomalies which are described here.
CAUSES
Abnormal small optic disk
Aplasia
Abnormal shape of the optic disk
Bergmisters papillae
Optic nerve head drusen (Fig. 13.1)
Myelineated nerve fibers (Fig. 13.2)
Optic disk coloboma
Congential Optic Nerve Anomalies 151
Optic disk pit

Morning glory syndrome
Tilted disk.
APLASIA
Aplasia is a rare anomaly characterized clinically by a total blind eye
with an afferent pupillary defect, an absent optic disk and an absent
retinal vasculature.
HYPOPLASIA
The optic disk hypoplasia is a sporadic condition. The affected can be
micro-ophthalmic or of normal size and usually exhibit a wide range
of visual impairment from normal vision to severe visual loss with
strabismus or nystagmus in bilateral cases. Visual acuity is determined
primarily by the integrity of the papillomacular bundle. The visual
fields show a localized defect. The disk is surrounded by a peripapillary
halo, bordered by a dark pigment ring called as the double ring sign.
Retinal vascular tortuosity is commonly seen. Histologically optic
nerve hypoplasia is characterized by a subnormal number of optic
nerve axons with normal mesodermal elements and glial supporting
tissue.
Some of the optic nerve hypoplasia are segmental. A
pathognomonic superior segmental hypoplasia with an inferior visual
field defect occurs in some children of insulin dependent diabetic
mothers. In cases of homonymous hemianopic hypoplasia, there is
congenital cerebral hemiatrophy.
Patients with periventricular leukomalacia often have a unique form
of optic nerve hypoplasia characterized by an abnormally large cup
and a thin neuroretinal rim contained within a normal sized disk can
be associated with intracranial and facial anomalies like septo-optic
dysplasial De Morsiers syndrome, congenital hypopitutarism,
hydrancephaly, arrhinencephaly, aniridia, homonymous optic
hypoplasia associated with congenital hemispheric aplasia, cyclopia,
enchelomeningocele and hypertelorism.
De Morsiers syndrome refers to the constellation of small anterior
visual pathways, absence of septum pellucidum, and agenesis or
thinning of the corpus callosum. MRI is the optimal noninvasive
neuroimaging modality for delineating congenital CNS malformations
in patients with septo optic dysplasia. In bilateral optic nerve
hypoplasia the coronal MRI shows diffuse thinning of the optic
chiasma.
The detection of hypopitutarism is an essential component of the

evaluation of children with optic nerve hypoplasia, because children
with endocrinological deficiency are at risk for impaired growth,
hypoglycemia, developmental delay, seizures and death. Parents
should be asked about the history of protracted neonatal jaundice
and previous episodes of hypoglycemia in the neonatal period.
Infants with optic nerve hypoplasia have superimposed delayed
visual maturation. Therefore infants who initially appear to be blind
may have improvement of their vision during the first several months
of life.
Treatment
Superimposed ambylopia should be treated with a trial of occlusion
therapy children with hypopitutarism should be treated with pituitary
hormone replacement.
BERGMISTERS PAPILLAE
The glial sheath of Bergmister envelops the posterior third of the
hyaloid artery it begins to atrophy about the seventh month of
gestation, even before the main vessel atrophies. The extent of the
atrophy is below the surface of the disk. If the atrophy at the disk is
less complete a tuft of glial tissue may be seen thorough out the life
over the optic disk called the Bergmisters papillae.
MYELINEATED NERVE FIBERS

Medullation or myelineation of the optic nerve begins in the fetal life
from the lateral geniculate body towards the globe. Normally the
myelination is completed shortly after birth at which time the myelin
sheath reaches the posterior aspect of the lamina cribrosa. The
medullated fibers may be seen starting from the disk and extending
towards the periphery (Fig. 13.2). Fundus examination shows irregular
feather-like patches which may or may not obscure the retinal blood
vessels. Rarely, isolated peripheral patches of myelination may also
occur.
Myelination of the nerve fibers results in visual field defects.
Myopia, coloboma, polycoria, keratoconus, oxycephaly and
neurofibromatosis have been associated with myelineated nerve fibers.
OPTIC NERVE HEAD DRUSEN

Deposition of hyaline like calcified material within the substance of
the optic nerve head.Optic nerve head drusen (Fig. 13.1) can be
inherited or can be associated with heredodegenerative conditions

like retinitis pigmentosa or angioid streaks or can be following long
standing papilledema, papillitis, vascular occlusions.
Clinically has an irregular, nodular, mulberry like appearance of
the surface of the disk. The physiological cup can be absent but venous
pulsation is present there can be abnormal tortous, anomalously
branching vessels. Disk can be pallor sometimes but will have irregular
margins. They vary greatly in size, shape and number, smaller ones
often coalesce to form larger ones.
The differential diagnosis of optic disk drusen includes
papilledema with which it is often confused. Fluorescein angiography
helps to differentiate between both the conditions which closely
simulate each other. Drusens exhibit the phenomenon of
autofluorescence and may stain during the late stages of angiogram.
They exhibit no leakage of fluorescein from the optic nerve head which
is usually present in papilledema.
Patients with disk drusen usually remain asymptomatic although
few cases have been reported to develop peripapillary and macular
hemorrhage. Drusens may directly compress the nerve fibers within
the disk and cause various visual field defects like enlargement of the
blind spot, arcuate scotoma or peripheral field constriction. Central
visual acuity is usually good.
COLOBOMA OF OPTIC DISK

A coloboma of the optic disk (Fig. 13.1) results from incomplete closure
of the embryonic fissure. The fissure initially closes in the middle and
then extends anteriorly and posteriorly until a small crescent at the
posterior pole remains open. When the lips of the fissure fail to fuse,
typical colobomas result. The coloboma of the optic nerve may occur
alone or may be associated with coloboma of the iris, retina, choroid
or ciliary body.
It usually occurs in two forms.
Optic Disk Coloboma in Association

with Retinochoroidal Coloboma
This form of coloboma is more common and frequently occurs
bilaterally. It is characterized by a large excavation which is usually
situated inferiorly with the normal appearing disk tissue pushed
superiorly. It is associated with superior visual field defects.
The retinochoroidal coloboma may involve the optic nerve
completely or occasionally there is a patch of healthy tissue between
Fig. 13.1: Optic nerve head drusen
the retinochoroidal coloboma and the optic disk coloboma. Such

colobomas are known as Bridge colobomas.
Coloboma of the Optic Nerve Entrance

These are isolated colobomas of the optic nerve wherein the disk
shows an enlarged and excavated nerve head with an expanded scleral
canal. It is usually unilateral and is associated with high myopia and
amblyopia. The central area of the nerve shows persistent hyaloid
remnants. The blood vessels which are believed to be cilioretinal in
origin emerge like the spokes of a wheel in a radical fashion from the
rim of the excavation. This is known as Morning glory syndrome (Fig.
13.2).
Fig. 13.2: Myelineated nerve fibers

Occasionally mild failure of closure of embryonic fissure leads to

the development of inferior crescent which is situated at the lower
edge of the disk. It closely resembles the myopic crescent and is found
to occur more commonly in hypermetropic and astigmatic eyes.
MORNING GLORY SYNDROME

The morning glory anomaly is a congenital, funnel shaped excavation
of the posterior fundus that incorporates the optic disk. The disk is
markedly enlarged, orange or pink in color and typically situated
within a funnel shaped excavation. Surrounding the excavation is a
variably elevated annular zone of altered retinal pigmentation. A white
tuft of glial tissue overlies the recessed central portion of the lesion.
The blood vessels appear increased in number and arise from the
periphery of the disk. They run an abnormally straight course over
the peripapillary retina and tend to branch at acute angles. It is often
difficult to distinguish arterioles from venules. The macula may be
incorporated into the excavation called as macular capture. Computed
tomography shows a funnel shaped enlargement of the distal optic
nerve at its junction with the globe.
Visual acuity anges from 20/200 and finger counting. Morning glory
syndrome is more common in females and rare in blacks.
Serous retinal detachment is the most noted complication of this
anomaly 26 to 38 percent of the eyes with morning glory result in
retinal detachment.
Associated with basal encephalocele with midfacial anomalies
(hypertelorism, cleft lip, cleft palate, depressed nasal bridge, midline
upper lid notch).
MRI is indicated in patients with mid facial anomalies and
neurological deficits because these patients are at high risk for an
associated basal encephalocele.
Treatment
superimposed ambylopia should be treated with a trial of occlusion
therapy. Patients with basal encephalocele should be evaluated for
surgical repair.
TILTED DISK
Tilted disk is caused by an oblique insertion of the optic nerve into
the globe. The upper temporal portion of the disk often lies anterior
to the lower margin. The vertical axis of the disk is directed obliquely
which gives it an oval appearance. This condition may be associated

with visual field defects which involve the upper temporal quadrant
because of the ectasia of inferonasal portion of the fundus. The visual
fields in the congenital tilted disk does not respect the vertical meridian
and it usually crosses the vertical meridian. Can be associated with
the situs inversus of the blood vessels.
Tilted disk is often found to be associated with degenerative high
myopia wherein scleral ectasia or staphyloma involve the posterior
pole temporal to the disk. This results in an oblique exit of the optic
nerve.
OPTIC DISK PIT

Optic disk pit is usually seen as a small excavation along the temporal
border of the disk covering nearly one-third of the surface of the
disk. It is usually round or oval in shape and appears darker than the
surrounding disk tissue probably because of the inability to illuminate
these small deep excavations. It is usually unilateral and the disk may
appear larger as compared to the fellow eye.
In 15 percent of the cases it can be bilateral. A cilioretinal artery is
found in 59 percent of the eyes with optic disk pit.
Approximately 45 percent of the eyes with congenital optic disk
pit develop serous macular elevations.
The macula demonstrates the following progression of events.An
inner layer retinoschisis cavity initially forms in direct communication
with the optic pit an outer layer macular hole develops beneath the
boundaries of the retinoschisis cavity.
An outer layer retinal detachment develops around the macular
hole. This outer layer detachment ophthalmoscopically can be mistaken
for an pigment epithelial detachment, but it does not hyperflouresce
on FFA.
The outer layer detachment eventually enlarges and obliterates
the retinoschisis cavity. At this stage, it becomes clinically
indistinguishable from a primary serous macular detachment.
BIBLIOGRAPHY
2. David J Apple, Maurice F. RABB (3rd ed) Ocular Pathology Mosby.
Jaypee, India 2003.
14 Optic Nerve Tumors
Nick Mamalis, Garrett Smith
INTRODUCTION
Tumors of the optic nerve are relatively rare lesions. However, these
lesions have significant risk for visual morbidity as well as other
problems related to the central nervous system (CNS). Optic nerve
glioma (astrocytoma) is the most common intrinsic tumor of the optic
nerve. Juvenile pilocytic astrocytomas are by far the most common
optic nerve tumor of children. Malignant gliomas of the optic nerve
occur much less frequently and are seen in adults. Meningiomas of
the optic nerve sheath are the second largest group of tumors which
may affect the optic nerve and occur more commonly in adults. Lastly,
secondary tumors of the optic nerve may arise from direct invasion
from intraocular malignancies, meninges, adjacent structures, as well
as distant metastases.
OPTIC NERVE GLIOMAS

Gliomas (juvenile pilocytic astrocytoma) are the most important optic
nerve tumor of children, accounting for 65 percent of all intrinsic optic
nerve tumors. 1 Gliomas are benign neoplasms arising from the
neuroglia (astrocytes and oligodendrocytes). The majority of optic
nerve gliomas are of astrocytic origin. However, a few rare optic
nerve gliomas arise from oligodendrocytes. The descriptive term
juvenile pilocytic astrocytoma is often used to describe this low-grade
glioma. Gliomas grow slowly, but can spread under the dura to invade
local structures. Patients typically present before the age of 20 with
progressive visual loss, proptosis, and disk pallor with or without
papilledema. Management includes observation, radiation, and
surgery.
Clinical Features
Age
In 1816 Antonio Scarpa first described optic nerve tumors and noted
that the majority of his patients with gliomas were children. This fact
has been substantiated in subsequent reports.1 In Duttons review of
gliomas of the anterior visual pathway, the mean age of presentation
was 8.8 years, with 70 percent of optic nerve gliomas occurring in the
first decade of life, 20 percent in the second decade, and 10 percent
between the ages of 20 and 80.1 Optic nerve gliomas are rare lesions
estimated at 5 percent of intracranial tumors and 3 percent of all orbital
tumors.2,3
Sex
Optic nerve gliomas show equal to a slight female preponderance.
One study of 594 patients had 295 (50 percent) females and 299 (50
percent) males.1 For gliomas confined to the optic nerve, earlier studies
suggest a slight female preponderance of about 2:1.4,5
Location
The size, growth pattern, and symptoms of the tumor depend upon
the location of the tumor. In a study of 1278 cases, 75 percent involved
the chiasm and optic nerve, while 25 percent were confined to the
optic nerve alone.1 Of the lesions involving the chiasm, 7 percent were
confined solely to the chiasm. Extension from the optic nerve into
intraocular structures, meninges, and brain occurs in a few rare cases.6,7
It is thus helpful to divide optic nerve gliomas into two categories:
orbital gliomas and intracranial or chiasmal gliomas.
Orbital gliomas vary in size and growth pattern, but are generally
slow growing benign tumors. Many orbital tumors slowly enlarge to
reach a plateau, then remain unchanged for many years. This
stabilization phenomenon is the reason that many considered these
as hamartomas. Hamartomas are a focal malformation resembling a
neoplasm, but is the result of faulty organ development composed of
an abnormal mixture of tissue elements.
Chiasmal gliomas seem to be more aggressive than orbital gliomas,
and a few cases have reported malignant change.8,9 Of the tumors in
the chiasm, 46 percent involved the hypothalamus or third ventricle,
interfering with hypothalamic and pituitary function.1 Thus, patients
with chiasmal tumors often present with endocrine abnormalities.
Optic Nerve Tumors 159
Chiasmal tumors are also a concern because they can obstruct the
third ventricle causing intracranial pressure elevation.
Presenting Signs and Symptoms

Orbital gliomas present with more orbital manifestations, while
chiasmal gliomas demonstrate more neurological symptoms. However,
both tumors share many of the same symptoms and many patients
present with tumors involving both the orbital optic nerve and the
chiasm.
Regardless of tumor location, patients may experience some degree
of unilateral visual dysfunction, visual field loss, afferent pupillary
defect, decreased ocular motility, optic atrophy, pain, headache, and
nystagmus. In Duttons review, 88 percent of patients presented with
vision loss, 26 percent presented with acuities between 20/20 and 20/
40, 19 percent between 20/50 and 20/200, and 55 percent were 20/
300 or worse.1 Visual field loss occurred in 63 percent of patients and
an afferent pupillary defect was seen in 75 percent of individuals.
Visual loss occurs due to astrocyte proliferation within the confines
of the dura and bone in cases of intracranial lesions. Initially, this
causes longitudinal axon bundle separation and nerve fiber
compression. The compression inhibits axoplasmic transport, with little
loss of axonal conduction. Further compression leads to demyelination
and mechanical disruption of axons. Intracranial gliomas are more
confined and compress the axon faster, producing quicker vision loss.
Orbital gliomas have more room to grow causing the characteristic
slow progressive visual loss, because only individual axons degenerate.
Spontaneous improvements in vision have been reported.10,11 This is
theorized from variations in mucoid substance and hydration, and
their effects on the optic nerve. In some cases rapid vision loss occurs
due to occlusion of the vascular supply. Gliomas affecting the optic
disk and retrolaminar portions of the nerve may compress the central
retinal vein producing optic disk swelling. The visual loss is rapid
and the clinical picture may simulate optic neuritis.
Proptosis is often the chief complaint of an orbital glioma in young
children, occurring 94 percent of the time in orbital lesions (Fig. 14.1).1
Because the tumor arises from the nerve within the muscle cone, the
proptosis is usually axial. Minimal proptosis is 2.0 to 4.0 mm ranging
up to severe proptosis at 10.0 mm or more.
Nystagmus is another initial sign of both orbital and chiasmal
gliomas occurring in 23 percent of patients.1 It may be vertical,
horizontal, seesaw, or rotary. Pain or headaches, and limited ocular
motility are other common symptoms of both types of gliomas.
Fig. 14.1: Child with an optic nerve glioma of the left eye demonstrating
proptosis with moderate inferior displacement of the left globe
On fundoscopic examination, 59 percent of patients demonstrated

some degree of optic atrophy and disk pallor (Fig. 14.2). Disk swelling
presents more frequently in orbital gliomas with a 48 percent occurrence
rate.1 In chiasmal tumors, disk swelling occurred 22 percent of the
time, and was often bilateral, due to increased intracranial pressure.
Optociliary shunt vessels are occasionally seen with optic nerve
gliomas, however they are far more common in optic sheath
meningiomas.
Other late and infrequent orbital glioma signs are venous stasis
retinopathy with iris neovascular glaucoma, anterior segment ischemia,
and hemorrhagic glaucoma from retinal vascular occlusion.
In chiasmal gliomas, 27 percent of patients had third ventricle
involvement causing increased intracranial pressure and 26 percent
reported hypothalamic or endocrine abnormalities.1,12 The endocrine
abnormalities included obesity, diabetes insipidus, panhypo-
pituitarism, dwarfism and precocious puberty.
Association with Neurofibromatosis

Several studies have published a 15 to 21 percent occurrence rate of
optic gliomas in neurofibromatosis patients. A study of 2186 published
cases of patients with optic gliomas demonstrated 29 percent of them
to have neurofibromatosis.1 Patients presenting to doctors with caf-
Fig. 14.2: Fundus examination of the same child

showing diffuse atrophy of the optic nerve
au-lait spots and diagnosed with neurofibromatosis, should have

regular ophthalmic examinations. Neurofibromatosis type 1 (NF-1)
patients commonly develop multiple CNS astrocytomas and these
lesions show a predilection for the orbital optic nerve and chiasm.13
NF-1 lesions tend to be multifocal and more extensive along the optic
pathways than in patients without NF-1. Patients with bilateral optic
gliomas usually have NF-1. 14 However, the incidence of visual
symptoms and progressive neurologic deficits was lower among those
patients with NF-1.
Radiographic Findings
On plain orbital radiographs 65 percent of optic gliomas can be
visualized mainly through enlargement of the optic canal.1 The classic
radiographic findings are enlargement of the optic foramen and J-
shaped excavation of the sella turcica. The optic canals are usually
symmetrical, and a 1.0 mm difference in the diameters or a vertical
height of 6.5 mm or more is considered pathologic.
Computerized tomography scans are more accurate, especially for
orbital lesions. The tumor usually appears as a well-defined spindle
or rounded shaped enlargement of the optic nerve.15 Kinking of the
nerve is a characteristic finding in orbital gliomas, due to elongation
of the nerve from secondary axial growth and downward
deflection.15,16
In evaluating anterior pathway lesions CT and Magnetic Resonance

Imaging are equivalent.17 However, for chiasmal, hypothalamic, and
optic tract lesions MRI is superior to CT, because in CT scans the
tumor images are isodense to brain tissue.17,18 MRI differentiates tumor
tissue from normal brain and neural structures better, thereby allowing
improved diagnosing and monitoring. However, microscopic extension
of the tumor cannot be detected. MRI has several other advantages
over CT. MRI does not expose young children to ionizing radiation,
allowing repeat serial examinations and it avoids scatter artifact near
bone.19
On T2-weighted images, gliomas are hyperintense compared to
normal optic nerve. Therefore, T2 weighted images is the best for
demarcation of tumor borders (Fig. 14.3).20 Arachnoidal gliomatosis
in neurofibromatosis patients can be visualized on T2-weighted axial
MRI studies as an area of high-signal intensity (due to a high water
content in the myxomatous tissue) surrounding a linear core of lower
signal intensity. 16,21 Gliomas in T1-weighted images are slightly
hypointense compared to normal optic nerve (Fig.14.4). T1-weighted
image is best for demonstrating tissue composition, characterizing
necrosis and mucinous degeneration.20
Histopathology
Gross Appearance
Optic nerve gliomas are typically contained within the dura (Figs 14.5A
and B). The dura is stretched and thin, but usually intact. Typical
gliomas appear tan to dusky red from the vascular congestion within
the tumor. Orbital gliomas are characteristically fusiform, with the
Fig. 14.3: Axial MRI scan of a patient demonstrating a diffuse, fusiform

enlargement of the optic nerve within the nerve sheath
Fig. 14.4: Sagittal MRI scan demonstrating sausage-like

enlargement of the optic nerve secondary to a glioma
Fig. 14.5A: Gross specimen demonstrating a relatively normal optic nerve on the
left with disuse enlargement of the nerve itself within the intact sheath secondary
to glioma
Fig. 14.5B: Low-power photomicrograph of the same patient demonstrating a

normal optic nerve to the left with an intact sheath around it. There is an enlargement
of the nerve itself secondary to the glioma with multiple large cystic spaces with
myxomatous type degeneration (hematoxylin-eosin 10)
borders of the tumor difficult to delineate. Thus, it is helpful to obtain

cross-sections at the end of each specimen.22 Gliomas may also invade
the arachnoid and pia, and extend through the subdural space. This
pattern occurs more often in neurofibromatosis patients.23 The nerve
itself may be of normal thickness, but the overall diameter may be
increased because of the perineural component. Cross-sections through
the middle portion show the whitish nerve enlarged and surrounded
by a cuff of arachnoidal tissue, which is then covered by stretched
dura.
Microscopic Findings
Most optic nerve gliomas consist of elongated, spindle-shaped,
pilocytic (hair-like) astrocytes (Fig. 14.6). Some researchers thus use
the term juvenile pilocytic astrocytoma to differentiate them from
other intracranial astrocytomas in older patients.24 These astrocytes
have a benign histological appearance rarely demonstrating mitotic
figures or malignant degeneration.22 The nuclei are usually uniform
and oval with some being hyperchromatic (Fig.14.7). The cytoplasm
is extended and contains glial filaments visible with special stains
such as GFAP.25 These spindle-shaped astrocytes are fairly cohesive
and damage the optic nerve by forming intersecting bundles that cause
axon separation or compression of the nerve.
The most distinctive and frequently encountered degenerative
change found in optic nerve gliomas is the Rosenthal fiber.22 Rosenthal
fibers are elliptical eosinophilic swellings found within astrocyte cell
processes and surrounded by hyalinized connective tissue (Fig.14.8).
These fibers consist of electron-dense granular material and glial
Fig. 14.6: Low-power photomicrograph of a juvenile pilocytic astrocytoma

(hematoxylin-eosin 100)
Fig. 14.7: Moderate power photomicrograph demonstrating a low-grade

astrocytoma of the optic nerve (hematoxylin-eosin 200)
Fig. 14.8: Moderate power photomicrograph demonstrating multiple eosinophilic

staining cytoplasmic inclusions consistent with Rosenthal fibers (hematoxylin-
eosin 250)
filaments. Foci of calcification from axonal debris commonly appear

in the fiber.
Vascular proliferation and atypia are frequently seen. These vessels
are located either in the pial septa or between bundles of astrocytes.
Periodically, enlarged congested sinusoidal vessels are encountered,
but hemorrhagic necrosis rarely occurs.
Pale staining areas that appear microcystic on hematoxylin-eosin
staining are frequently interspersed among the astrocytes. With special
stains, the microcystic spaces can show mucosubstance (myxomatous
glioma) from mucin-producing cells that are present in the area. The
microcysts are not intracellular but are extracellular accumulations
of a mucicarmine-negative mucoid substance that stains with periodic
acid-Schiff (PAS) and acid mucopolysaccharide. It is believed that
astrocytes produce this mucoid hydrophilic material that also
contributes to tumor enlargement.22
Older gliomas become fibrotic with lipoidal histiocytes and thick-
walled fibrotic blood vessels suggestive of an angiomatous lesion,
thus, making older gliomas more difficult to recognize.
Optic nerve gliomas have two distinct growth patterns: perineural and
intraneural. 26 In the perineural pattern, more of the perimeter
astrocytes proliferate to widen the epipial-subarachnoid space within
the intact dura while thus compressing the residual optic nerve as a
central band. This circumferential tumor tissue consists mostly of
proliferating astrocyte nests, intermingled with meningothelial cells,
fibroblasts, and fibrovascular arachnoidal trabeculae, with mucinous
and microcystic degeneration. Studies by Stern et al demonstrated
these findings and they proposed the term arachnoidal gliomatosis
(Fig.14.9). 26 Perineural growth often involves more of the optic
pathways, because the perimeter astrocytes proliferate and can tunnel
along the nerve under the dura. This perineural growth is associated
with neurofibromatosis type 1 patients.16,26 One study observed 94
percent of glioma patients with perineural growth also had
neurofibromatosis.16
The intraneural growth pattern predominates in patients without
neurofibromatosis.26 In this pattern, the optic nerve enlarges instead
of being compressed. Intra-axial astrocytes proliferate causing
Fig. 14.9: Low-power photomicrograph demonstrating proliferation of the low-

grade astrocytoma from the optic nerve to the area underlying the sheath
demonstrating arachnoid gliomatosis from a patient with neurofibromatosis
expansion of fibrovascular trabeculae with slight cystic degeneration.

The increasing nerve diameter crushes the subarachnoid space and
fuses the pia mater to the arachnoid and dura.1
In both types of growth patterns the optic tumor enlarges by
proliferation of neoplastic glial cells, accumulation of extracellular PAS-
positive mucosubstance secreted by the astrocytes, reactive gliosis,
meningeal hyperplasia, and congestion within dilated blood vessels.
Growth is usually slow, but accelerated expansion can result from
cystic degeneration or intralesional hemorrhage. The majority of
gliomas are benign and enlarge by bulk local growth causing
demyelination and compression of optic nerve fibers. However,
gliomas are true neoplasms and can tunnel under the dura extending
along optic tracts and infiltrate the leptomeninges or intraocular
structures.9,27 A few rare gliomas can undergo malignant evolution
and spread throughout the cerebrospinal fluid.28,29
Gliomas and meningiomas of the optic nerve usually produce
similar symptoms. Therefore, identifying which type of lesion the
patient has is important. Current imaging and ultrasound techniques
have become excellent at distinguishing the type of lesion the patient
has, but when there is doubt many clinicians still advocate biopsy.
However, even on biopsy confusion may arise. Possible reactive
proliferation of meninges overlying the glioma making possible the
misdiagnosis of meningioma if a very superficial biopsy of the optic
nerve is done.
Management
Defining clear-cut guidelines for correct management of optic gliomas
is difficult, because the natural course of gliomas is variable. Reported
statistics and treatments results vary considerably causing much
controversy to exist over the proper management of optic gliomas.
A study by Wright and McDonald30 showed that in half of their
patients, the tumor appeared to stop growing without treatment. It is
thought that in this group the tumor was stable upon presentation or
slowly enlarged to reach a plateau remaining unchanged for many
years. In the other half of the patients, the glioma continued growing,
resulting in clinical signs and symptoms that required surgical removal.
This study shows the dilemma of whether to surgically intervene
causing blindness in that eye or to just follow the tumor
radiographically and maintain partial vision. If the tumor appears
stable it is worth watching, but if the tumor progresses intracranially,
it can be deadly.
If a patient presents with an optic glioma that appears to be fairly

asymptomatic and confined to the orbit, current thought is to follow
the patient without treatment.1 Because many studies have shown
approximately half the gliomas plateau and remain dormant, with
some cases of vision actually improving later on.30,31 Serial CT or MRI
scans, pupillary reactions, visual acuity, and visual field examinations
should be done to monitor tumor growth. If the tumor enlarges to
cause blindness, severe proptosis, or pain, then complete removal by
lateral orbitotomy is warranted.32 The risk of just monitoring gliomas
is that some can spread throughout the CSF invading distant areas. A
few rare gliomas undergo malignant evolution.8 Kocks reported a
child who developed lumbar spinal metastases from a chiasmal
glioma.33 However, the majority of gliomas grow slowly over months
to years and spread by local enlargement. If on radiographic scans,
the tumor shows extension along the intracranial portion of the nerve
and threatens the chiasm, then surgery is also recommended. Once
the tumor extends to the optic chiasm the risk of death rises to about
28 percent.1 Surgical intervention at this point does not improve
survival, and has significant visual morbidity and potential mortality.
Some research suggests a short-term benefit from radiotherapy in
doses exceeding 4500 cGy for chiasmal and midbrain tumors,11,34 but
overall survival and ultimate recurrence show no benefit to
radiotherapy.1,35 This raises the question as to whether radiotherapy
is worth the side effects, because radiation in children has many
permanent adverse effects on the CNS and endocrine function. 36
Among 511 patients treated with radiotherapy and followed for 10
years, 69 percent demonstrated stable vision, 42 percent showed tumor
progression, and 28 percent died from the disease. In contrast to the
treated group, 203 similar patients were followed without treatment
and showed comparable results; 77 percent demonstrated stable vision,
42 percent showed tumor progression, and 29 percent died.1 In a
study by Packer et al they advocated that chemotherapy could
significantly delay the need for radiation in children.14 Yet, there is
little published data on the role of chemotherapy.
Although optic gliomas are benign neoplasms they can result in
significant morbidity and mortality. Therefore, the clinical approach
to these tumors must be vigilant, with attentive observation and
aggressive intervention when necessary.
MALIGNANT GLIOMAS OF THE OPTIC NERVE

Malignant astrocytomas of the optic nerve are a rare, but aggressive
and deadly disease. The tumor arises from malignant astrocytes
located in intracranial optic pathways and rapidly spreads to invade
numerous structures. Patients typically are middle-aged adults that
present with decreased vision, visual field loss, retro-orbital pain,
and disk swelling. Most patients progress to blindness and death
within a year.37
Clinical Features
In contrast to benign gliomas that occur in children, malignant gliomas
are a disease of middle-age. In Duttons review the average of
presentation was 47, with patients ranging from six to eighty. 1
Malignant optic gliomas show a male preponderance, with 65 percent
occurring in males, and 35 percent occurring in females.
Malignant gliomas of the optic nerve arise from malignant
astrocytes that originate in intracranial optic pathways.37 Rarely, it
arises in the orbital optic nerve. Malignant gliomas rapidly spread
anterior and posterior to involve the optic nerve, chiasm, optic tracts,
hypothalamus, third ventricle, thalamus, temporal and occipital lobes.
The clinical course is unilateral visual loss that progresses to
blindness and death in an average of 11 weeks, but can range up to 60
weeks.1 The malignant astrocytes typically attack one side, then
rapidly spread through the chiasm to involve both optic nerves. At
presentation 64 percent of patients have bilateral visual loss. The final
visual acuity reported in a study of 22 patients showed that in the less
affected eye only 23 percent had vision of 20/400 or better, while 63
percent were NLP. In the more affected eye 86 percent were NLP.
Visual field defects occurred in 94 percent of patients.1
On initial presentation normal optic disks are often seen, but within
weeks the disk progressively swells. If the patient lives long enough
optic atrophy ensues.
Malignant gliomas typically arise intracranially or in the chiasm
and generally affect intracranial optic pathways. Thus, neurological
symptoms are more common than proptosis. Neurological signs
include convergence and gaze abnormalities, paresthesias, partial
ophthalmoplegia, seizures, confusion, and hallucinations.
Hypothalamic involvement usually occurs in the final stages and causes
many of the deaths.37
Retro-orbital pain is a common symptom. The visual loss and retro-

orbital pain often lead to an initial diagnosis of optic neuritis. Rapid
and progressive visual loss should include radiological imaging.
Radiology
Plain orbital radiographs rarely reveal malignant gliomas.
Computerized tomography provides a 79 percent chance of disclosing
the tumor on initial examination.1 Images portray an enlarged chiasm
and optic nerves. Few reported cases have used MRI, but MRI has
revealed the tumor in all cases.38,39
Pathology
Histological examination is consistent with malignant astrocytoma
showing atypical pleomorphic astrocytes with numerous mitotic
figures. Secondary vascular and endothelial proliferation can also be
found.40 The malignant cells encircle and compress the optic nerve
inhibiting axoplasmic transport and capillary perfusion, causing
demyelination and axonal degeneration. The neoplastic cells usually
extend under the pia along the optic pathways or directly within the
brain substance. The tumor can spread to invade the orbit,
hypothalamus, third ventricle, basal ganglia, or intraparenchymal
brain.
Prognosis
Malignant gliomas are a sad and devastating disease with the overall
mortality rate of 97 to 100 percent with a mean survival of 8.7 months
following diagnosis.1 Some patients treated with 5000 to 6000 cGY
radiotherapy showed temporary visual acuity improvements and
slightly prolonged life, but ultimately died from the disease. Advances
in cancer research will hopefully led to better treatments.
OPTIC NERVE MENINGIOMAS

Meningiomas are the second most common optic nerve tumor, after
gliomas. 41 Meningiomas are benign neoplasms arising from
meningothelial cells typically in the arachnoid. Patients generally are
middle-aged adults and present with decreased vision, visual field
loss, proptosis, disk atrophy, disk swelling, and later on optociliary
shunt vessels. Meningiomas grow slowly, but are invasive and
infiltrate surrounding structures. Management includes conservative
monitoring, radiotherapy, and surgery.
Clinical Features
In contrast to gliomas, meningiomas occur later in life. In Duttons
review of optic nerve meningiomas, a study of 256 patients
demonstrated the average age of presentation to be 40.8 years.41 The
average age for males was 36, and the average age for females was
42. Even though the average age for females was older, meningiomas
show a slight female preponderance at 61 percent female and 39 percent
male. Approximately 4 to 10 percent of meningiomas occur in children
and tends to be more aggressive.22,41 Therefore, meningiomas should
still be included in the differential diagnosis of a lesion causing
proptosis and progressive visual loss in a child. Similar to gliomas,
there is a proven higher incidence of meningiomas in patients with
neurofibromatosis.42
Researchers classify optic nerve meningiomas into three types
based on tumor origin. Primary tumors if they originate from the
meninges in the optic nerve and secondary tumors if they originate
from cranial meninges and then extend into the orbit. Approximately
90 percent of meningiomas affecting the optic nerve are secondary,
extending from the olfactory groove and sphenoid ridge.43,44 A third
rare type, ectopic (extradural) arise from congenitally displaced
meningothelial cells along the floor or roof of the orbit.
Signs and Symptoms

The signs and symptoms of meningiomas depend upon the origin
and location of tumor growth. For ease of understanding, meningiomas
affecting the eye can be divided into four groups depending upon
location: (i) dura restricted, (ii) orbital, (iii) intracanalicular, and
(iv) secondary. The natural course of meningiomas is unpredictable,
because they can invade any surrounding structure. Therefore,
different tumors share many of the same clinical and pathologic signs.
Ninety-five percent of primary meningiomas have unilateral
involvement, but 5 percent are bilateral.41 The bilateral meningiomas
typically arise within the optic canal or chiasm (intracanalicular).41,43
The most common symptom of all meningiomas is gradual vision
loss occurring over one to five years. A study of 380 patients
demonstrated 96 percent to have decreased vision; with 45 percent
presenting with acuities between 20/20 and 20/40, 31 percent between
20/60 and 20/400, and 24 percent with counting fingers or worse.41,45
The second most common symptom was visual field loss occurring
in 83 percent of patients.41,46 Peripheral constriction was the most
characteristic visual field loss. Central, centrocecal, paracentral
scotomas, and increasing blind spot visual field losses also occurred.
Decreased color vision was reported in 73 percent of the patients.
Early or rapid vision loss occurs in dura-restricted meningiomas. These
meningiomas grow similar to a glioma, and remain confined within
the dura. As the meningothelial cells proliferate within the subdural
spaces, the tumor begins compressing the optic nerve and inhibits
axoplasmic transport. Further compression leads to demyelination
and mechanical disruption of axons. Similarities to gliomas also occur
when the meningioma infiltrates the nerve and widens the septa.
More characteristic slow visual loss occurs when the meningioma
penetrates the dura and grows outside of the dura (orbital
meningiomas). In this situation, meningothelial cells proliferate for
many months to years within the orbit and the tumor becomes very
large before compressing the optic nerve. The tumor begins to slowly
push on the posterior pole of the globe causing axial proptosis,
hyperopia, and striae. Axial proptosis is often the presenting symptom
of meningiomas and occurred in 59 percent of the study patients.41 In
this situation, when the tumor enlarges outside the dura, it can impinge
upon the extraocular muscles limiting ocular motility. Forty-seven
percent of study patients complained of limitation of ocular motility.
The tumor infrequently encroaches on one of the cranial nerves, causing
cranial nerve palsy.44 In dura-restricted meningiomas, as the tumor
enlarges it impinges and stiffens the optic nerve causing a mechanical
restriction of extraocular muscle function.
Disk swelling is an early finding in 48 percent of patients of all
types.41 Disk swelling occurs due to compression of the central retinal
vein and meningeal vasculature or spread of tumor cells to the anterior
end of the perineural space, with interference of disk circulation. As
compression of the optic nerve progresses the incidence and degree
of optic atrophy increases. Forty-nine percent of patients progress to
develop optic atrophy.
The classic pathognomonic triad for meningiomas of gradual
unilateral vision loss, optic atrophy, and optociliary shunts (Fig. 14.10)
occurs in 30 percent of patients.41 Imes et al47 followed the development
of optociliary shunts over eight and a half years in a woman with an
optic nerve meningioma. The first two years the woman had chronic
disk swelling and congestion of the central retinal vein. After two
years, Imes observed the dilation of regressed, but vestigeal,
retinociliary anastomoses that were present in earlier embryonic
development. The prolonged inhibition of retinal vein circulation
re-established the flow of blood from retinal veins through optociliary
shunts into the choroidal circulation. Then as the optic atrophy
worsened in the woman, the shunts regressed over the years.
Fig. 14.10: Fundus photograph of the optic nerve from an elderly female patient
demonstrating optociliary shunt vessels with atrophy of the nerve
Meningiomas affecting the optic canal or chiasm (intracanalicular)

may simulate atypical retrobulbar neuritis with decreased visual acuity
and optic atrophy. Proptosis and disk edema are rarely seen. Tumors
in the sphenoid ridge may also affect the nerves to the extraocular
muscles resulting in strabismus as a presenting sign.
Radiology
Plain orbital radiographs rarely see meningiomas. Cases of
hyperostosis of neighboring bone will show up, but these are rare.48
Computerized tomography has revolutionized diagnosing optic nerve
tumors, with visualization of 97 percent of meningiomas.41 CT scans
should be obtained before and after iodinated contrast medium
infusion. Thin sections (1.5 to 3 mm) should be taken to demarcate
tumor edges. Dura-restricted meningiomas often appear as a well-
defined smooth tubular enlargement of the optic nerve.48 The majority
(64%) of meningiomas shows this diffuse tubular thickening of the
optic nerve.49 Orbital tumors growing outside the dura show globular
perioptic or irregular and serrated enlargement, unlike dura-restricted
tumors that demonstrate a fusiform shape.48,49 The dura-restricted
tumors are commonly confused with gliomas, because they both are
ensheathed by the dura. Helpful findings are calcifications, which are
usually present in meningiomas and not typically found in gliomas.50
Another important radiographic sign helpful in diagnosing
meningiomas is tram tracking.41 In tram tracking the optic nerve can
be seen as a central black line through the whitish mass (Fig. 14.11).
Tram tracking, however, may also be visualized in inflammatory
Fig. 14.11: CT scan of the eye and orbit showing a diffuse meningioma of the
nerve anteriorly with the tram-track sign
perioptic pseudotumor and other diffuse sheath thickenings.51 In

coronal views the denser and thickened optic nerve sheath appears
as a dense circle around the more radiolucent optic nerve.
Magnetic resonance imaging offers more precise and sensitive
detection of intracanalicular or intracranial extension of meningiomas
than CT.48,52 T1-weighted images should include fat-suppressed fine-
cut axial and coronal images with and without gadolinium enhance-
ment. The T1-weighted images disclose the characteristic tram-track
appearance of the optic nerve in meningiomas. Axial and coronal T2-
weighted images provide the most sensitive method of determining
the extent of tumor involvement.
An additional imaging technique called OctreoScan is used to
support and follow the diagnosis of meningiomas. OctreoScan
(Indium-111 pentetreotide) is a radio-labeled ligand that binds to the
somatostatin receptors in meningiomas. The binding is highly
sensitive, but not very specific because other classes of tumors also
bind somatostatin. OctreoScan is therefore helpful in following tumor
progression in cases of observation or tumor treatment response.52
Histopathology
Meningiomas can arise from any of the different cells that comprise
the meninges (Fig. 14.12). However, current researchers believe the
majority of meningiomas arise from the meningothelial cap cells found
in arachnoid villi.41,42 Arachnoid villi are smaller and similar to arach-
noid granulations (grape-like tufts of arachnoid that penetrate dural
venous sinuses and affect transfer of CSF to the venous system).
Arachnoid villi are collected along the intraorbital and canalicular
Fig. 14.12: Gross photograph of an optic nerve sheath meningioma
portions of the optic nerve and also along the sphenoid ridge,
tuberculum sellae, olfactory groove, and other areas of the meninges.
Neoplastic meningothelial cap cells are spindle or oval shaped and
form densely packed concentric whorls with psammoma bodies (Figs
14.13A and B). Psammoma bodies are calcifications that develop in
the whorl centers from hyalinization and deposition of calcium salts
(Figs 14.14A and B). This pattern is the meningothelial pattern and is
the most idiosyncratic degenerative change found in meningiomas.
Meningiomas rarely show mitotic figures or malignant degeneration.22
Cells from the arachnoidal trabeculae of the meninges are of
mesodermal origin and can proliferate to cause fibroblastic
meningiomas. This type may metastasize. 41 A combination of
meningothelial and fibroblastic is called the transitional pattern.
Meningiomas are benign neoplasms that grow slowly over months to
years. Similar to gliomas, meningiomas can tunnel in the subdural
spaces traveling along the optic pathways and infiltrate intraocular
structures. However, unlike gliomas, meningiomas are invasive and
can penetrate the dura to invade adjacent orbital structures, such as
the extraocular muscles, sclera, or bone.22 Optic nerve meningiomas
arising in the orbit can spread posteriorly through the optic canal to
the chiasm or into the middle cranial fossa. At present, it is thought
that meningiomas do not invade the brain or pituitary, and it appears
meningiomas have little effect on the pituitary-hypothalamic axis
or increasing ICP. 42,44,53 Meningiomas can infiltrate the bone by
entering the haversian canals and initiate hyperostosis and bony
proliferation.54
Fig. 14.13A: Low-power photomicrograph demonstrating nests or whirls of

meningothelial cells underlying the optic nerve sheath which is to the left
Fig. 14.13B: Medium-power photomicrograph of a meningioma showing the spindle

cells with a concentric whirl-type arrangement in the center (hematoxylin-eosin
200)
Fig. 14.14A: Medium-power photomicrograph of a meningioma demonstrating

cells with a round nucleus and an eosinophilic staining cytoplasm with a small
psammoma body in the center (hematoxylin-eosin 200)
Fig. 14.14B: Higher power photomicrographs showing a large, calcified,

hyalinized, psammoma body (hematoxylin-eosin 250)
Management
The management of optic nerve meningiomas is controversial because
their course is unpredictable. Traditional management includes
observation, radiotherapy, and surgery. Traditionally, if a patient
presents with the tumor confined to the orbit and visual acuity better
than 20/40, observation was recommended.46 Observation includes
ophthalmic examinations and CT or preferably MRI scans every six
months. If visual acuity is progressively lost below 20/40, the visual
field is constricting, or imaging scans show dangerous growth the
patient is encouraged to undergo radiotherapy for preservation of
vision.46,52 If the eye is totally blind and the meningioma confined to
the orbit then the patient can choose to monitor the tumor or surgically
excise it. If the meningioma is spreading posterior or enlarging and
causing proptosis then total removal should be performed.
Because of the characteristic slow growth and benign pattern of
primary optic nerve meningiomas, the overall tumor-related mortality
rate is 0 percent according to Duttons major review.41 This fact
coupled with the risks of radiation or surgery are the reasons many
researchers advocate observation in patients over 40. The disadvant-
ages to sole observation is the possible risk of tumor spread. Of 228
orbital lesions only 20 percent showed posterior extension.41 However,
tumors originating in the canal or chiasm have a 38 percent chance of
contralateral involvement.52
The most obvious disadvantage of simple observation is gradual
vision loss. Kennerdell et al showed that of 39 patients that did not
receive treatment of any kind, not one maintained good visual acuity
for more than four years.46 This is in striking contrast to patients
treated with radiotherapy where 73 percent of them had improved
vision.41
Radiotherapy is the most promising treatment modality because
of its ability to inhibit tumor growth and restore vision.46,55 Older
radiation treatments were less precise and exposed the chiasm,
contralateral optic nerve, and surrounding tissues to ionizing radiation,
causing optic neuropathy and secondary malignancies. Leber et al56
studied the dose to damage relationship of older modalities. They
found that the patients receiving less than 10 Gy per day had no
incidences of optic neuropathy. 26.7 percent of patients receiving 10
to 15 Gy developed sequelae and 77.8 percent of patients receiving
greater than 15 Gy developed sequelae. The newer conformal
radiotherapy is much more precise and attains an improved therapeutic
ratio. The accuracy of computer-guided stereotactic radiotherapy only
exposes the patient to 1.8 Gy.52 This explains why many current
researchers advocate radiotherapy as primary management in all optic
nerve meningiomas as well as adjuvant treatment for incompletely
resected tumors.46,55,57,58
A few successful therapeutic surgical interventions have been
reported in tumors confined to the anterior or middle third of the
optic nerve.46,52,59-61 However, most surgical intervention results in
blindness, typically attributed to ischemia of the optic nerve.46,52 In
blind eyes surgical excision of the tumor is warranted if the tumor
threatens, the optic canal, chiasm, or intraocular structures. Surgical
removal is also indicated in blind eyes if the enlarging tumor causes
pain or proptosis.
Although optic nerve meningiomas are benign neoplasms, they
can result in blindness. Many technological advances have helped
patients maintain their vision, but the clinical approach must be
attentive and when necessary aggressive treatment implemented.
SECONDARY OPTIC NERVE TUMORS

The majority of tumors involving the optic nerve are from secondary
malignancies. Only 18 percent of tumors arise within the optic nerve
itself, while 82 percent invade the nerve secondarily.62 There are four
main routes of invasion into the optic nerve: direct extension from
the eye, meninges, adjacent structures, and blood-borne metastatic
invasion via the ophthalmic artery. Ginsberg et al63 presented a review
of 117 cases of secondary optic nerve tumors. They found 39 percent
to arise from intraocular tumors, 33 percent from blood-borne tumor
seeding, 20 percent from meningeal tumors, and 8 percent invaded
from adjacent structures.
Extension from the Eye

The most common secondary optic nerve malignancy is from
intraocular structures. 62 Retinoblastoma and uveal melanoma
constitute the majority of secondary intraocular tumors.
Retinoblastoma has been known for many years to invade the optic
nerve, with 26.7 percent of patients with retinoblastoma in a mass
eye and ear study showing extension into the optic nerve.62 The tumor
is usually limited by the lamina cribrosa, but may extend past it to
invade the chiasm or brain.64 Predisposing factors to nerve invasion
include elevated intraocular pressure, glaucoma, tumor seeding into
the vitreous, and necrotic retinoblastoma.65 The prognosis significantly
worsens once the tumor progresses beyond the lamina. Kopelman
et al66 reported that the most dangerous risk factor of retinoblastoma

was penetration of the coats of the eye, and the second most significant
risk factor was the degree of optic nerve invasion.
Uveal melanomas invade the nerve less than retinoblastoma, with
6.5 percent of uveal melanomas invading the optic nerve.62 The diffuse
melanomas have a more malignant cell type than typical nodular
melanomas. Thus, diffuse melanomas are more aggressive at invading
the optic nerve and carry a worse prognosis. Juxtapapillary melanomas
can invade the nerve by direct extension causing hyperemia and disk
edema. The lamina cribrosa generally limits the tumor growth, but
posterior extension into the orbit, chiasm,65 and brain67 have been
reported. Weinhaus et al68 on univariate analysis found a direct
correlation between tumor death and the degree of optic nerve
invasion. Predisposing factors for invasion include elevated intraocular
pressure, juxtapapillary location, glaucoma, and necrotic tumors.
Blood-Borne Metastasis
Hematopoietic malignancies involving the optic nerve include
leukemia, lymphomas, and myeloma. In most forms of leukemia the
optic nerve and nerve head become infiltrated with abnormal white
blood cells. Children with acute lymphoblastic leukemia are the most
affected with 13 percent of patients having optic nerve invasion.69 In
384 autopsy specimens, Kincaid et al70 found 82 percent of patients to
have ocular involvement. Unilateral or bilateral visual loss is the most
common clinical symptom. Disk swelling, as well as pallor, splinter
hemorrhages from infiltration, and increased intracranial pressure
often occur. Histologically, perivascular and discrete tumor infiltration
can be seen. Combined intrathecal chemotherapy and localized
radiotherapy have improved visual function in some cases.71
A few cases of Hodgkins and Non-Hodgkins lymphomas
involving the optic nerve have been reported.62 Invasion into the optic
nerve occurs from both chronic systemic lymphoma and CNS
lymphoma that invades via the meninges. Multiple myeloma
occasionally invades the eye, but only one case of optic nerve invasion
has been reported.72
Solid tumors that metastasize to the eye and optic nerve are rare
and typically occur in parallel with widespread systemic metastasis.
In Ginsbergs review of primary metastatic sites to the optic nerve,
breast cancer was the most recurrent distant tumor at 33 percent.
Lung cancer followed second at 11 percent and stomach third at
6 percent. Others included pancreas 3 percent, mediastinum 3 percent,
skin 3 percent, melanoma 2 percent, uterine 2 percent, and ovarian at
2 percent.63
Extension from the Meninges and Brain

Neoplastic cells gain access to the optic nerve by anterior progression
through the optic canal via the subarachnoid space. Neoplasms
entering the nerve by this route include primary intracranial tumors
(meningial carcinomas, reticuloendothelial sarcomas) plus secondary
metastatic tumors to the CNS, which include lymphomas, melanoma,
and myeloma.65 The course of these neoplasms is variable depending
on the aggressiveness of the tumor. Slow growing tumors can spread
anteriorly producing papilledema and visual dysfunction after the
disease is advanced. Aggressive, rapidly proliferating neoplasms
invade the axonal bundles, causing early visual loss and disk edema.
The prognosis is poor once visual loss occurs from metastasis, with
survival less than two years. 73 Palliative treatment consisting of
chemotherapy and radiation has resulted in transient improvement
in visual function in some cases.74
Primary tumors from the CNS (ependymoblastoma, pituitary
adenoma) rarely invade the optic nerve, but will commonly compress
the optic nerve causing a neuropathy.62
Extension from Adjacent Structures

Tumors arising in the orbit, nasal sinuses, and nasopharynx generally
compress the optic nerve instead of invading it. Visual field loss,
proptosis, and pain are common presenting symptoms. Treatment
for orbital tumors involves radiation, which often causes optic
neuropathies.
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Abnormalities of
15 Optic Nerve Head
Reena M Choudhry, Saurabh Choudhry, Amar Agarwal
OPTIC ATROPHY
Optic atrophy is characterized by loss of conducting function of the
optic nerve, with an increase in pallor of the disk as a result of gliosis
and loss of capillaries of the disk.1-16 Optic atrophy results from injury
to any portion of the retino-geniculate pathway (from retinal ganglion
cells to lateral geniculate body).
Primary optic atrophy is caused by lesions which cause a death of
the axons of the optic nerve without causing a swelling of the
optic nerve. The lesions may affect the visual pathway from
retrolaminar portion of the optic nerve to the lateral geniculate
body. Causes of primary optic atrophy can be retrobulbar neuritis,
compression due to aneurysms or tumors, toxic and nutritional
neuropathies and trauma.
Secondary optic atrophy is caused by conditions, which produce
swelling of the optic nerve head. The causes include papilledema,
papillitis, and anterior ischemic optic neuropathy.
Consecutive optic atrophy is a result of retinal or choroidal disease
leading to destruction of ganglion cells. It could occur following
chorioretintis, Retinitis pigmentosa, pathological myopia, central
retinal artery occlusion and after pan retinal photocoagulation.
Cavernous (Glaucomatous) optic atrophy is caused by loss of nerve
fibers in advanced glaucoma. The neuroretinal rim is healthy in
glaucomatous optic atrophy in contrast to primary optic atrophy
with a large cup.
Segmental optic atrophy is usually seen in toxic and nutritional
neuropathies and ischemic optic neuropathies.
Hereditary optic atrophy may be congenital or associated with
Lebers optic neuropathy, Kejr syndrome, Behr syndrome and other
systemic syndromes like Friedreichs ataxia.
Fig. 15.1: Optic atrophy
Clinical Features
Primary optic atrophy shows chalky white disk (Fig. 15.1) with
sharply defined margins and normal appearing retinal vessels and
surrounding retina (Table 15.1).
Secondary optic atrophy shows dirty gray pallor of the optic nerve
head with poorly defined margins, obliterated cup, sheathing and
narrowing of arteries in the peripapillary area.
Consecutive optic atrophy shows waxy pallor of the disk with
marked attenuation of the arteries. Signs of associated retinal
pathology are present.
Cavernous optic atrophy shows a pale disk with pathological
cupping, thinning of the neuroretinal rim, nerve fiber layer loss
and laminar dot sign.
Segmental optic atrophy shows pallor of the temporal side or other
segments with sharply defined margins and no retinal pathology.
Relative afferent pupillary defect is present in unilateral and
asymmetric cases.
Color vision is reduced in correlation with the visual loss.
Visual fields show varied defects depending upon the cause of the
optic atrophy.
Kejr syndrome: This is an hereditary optic atrophy which is bilateral
and is autosomal dominant. It occurs between the ages of 4-10
years.
Behr syndrome: This is autosomal recessive and is another hereditary
optic atrophy condition. Occurring during the first 10 years of life.
Wolfram syndrome: This is another hereditary optic atrophy disease
and is also referred to as DIDMOAD = Diabetes Insipidus, Diabetes
Mellitus, Optic Atrophy and Deafness.
Abnormalities of Optic Nerve Head 187
Table 15.1: Differences between primary, secondary

and consecutive optic atrophy
Features Primary Secondary Consecutive
1. Disk color Chalky white Dirty grey Waxy pallor
2. Margins Well-defined Blurred Well-defined
3. Lamina Well-seen Not seen Well-seen
cribrosa
4. Retinal Normal Peripapillary Marked
vessels sheathing and attenuation
narrowing of of arteries
arteries.
Veins are
Tortous and
Walls are
Sclerosed
5. Surrounding Normal Edema Associated
retina retinal
pathology
6. Example Pituitary Papilledema, Retinitis
Tumor, optic papillitis pigmentosa,
Nerve tumor CRA occlusion
Foster-Kennedy syndrome: There is unilateral papilledema and

contralateral optic atrophy. It occurs due to frontal lobe tumors.
Pseudo Foster-Kennedy syndrome: In this there is optic atrophy in one
eye and a disk swelling in the other eye due to AION.
Differential Diagnosis
One should differentiate between primary, secondary and consecutive
optic atrophy (Table 15.1).
Optic Neuritis
Optic neuritis is defined as an inflammatory or demyelinating disorder
of the optic nerve characterized by sudden loss or diminision of vision,
associated with ocular pain and dyschromatopsia.1-16 Most common
cause of optic neuritis is demyelinating disorders like multiple sclerosis.
Viral (mumps, measles, herpes zoster, cytomegalovirus, or HIV),
bacterial (tuberculosis, syphilis or lymes disease) and other
(histoplasmosis,cryptococcosis,toxoplasmosis or toxocariasis)
infections can also cause optic neuritis. In children optic neuritis can
occur post-immunization. Other possible causes are adjacent paranasal
sinus inflammation, systemic collagen vascular diseases and intra
ocular inflammation.
Clinical Features
Decreased visual acuity ranging from 6/9 to no perception of light.
There is rapid worsening in next few days reaching maximum
deficit by 1-2 weeks. Recovery occurs over next 4-6 weeks.
Decreased color vision, which is more than the visual deficit.
Acquired optic nerve disease tends to cause red-green defects. An
exception occurs in glaucoma and in autosomal dominant
neuropathy which initially causes blue-yellow deficit. It has been
recently found that visual field loss in glaucoma is detected earlier
if perimetry is performed using a blue light stimulus on a yellow
background. Acquired retinal disease tends to cause blue-yellow
defects except in cone dystrophy and Stargardts disease which
cause a predominantly red-green defect.
Contrast sensitivity and stereoacuity is reduced.
Relative afferent pupillary defect (RAPD) is present in unilateral
or asymmetric cases.
Visual fields show central, centrocaecal or arcuate field defects.
Cells may be seen in the vitreous.
Fundus examination:
Retrobulbar neuritis: Optic disk appears normal (most common
presentation in adults).
Papilitis: The disc appears swollen and hyperemic (Fig. 15.2A),
associated with or without peripapillary flame shaped hemorrhages.
Cells in the posterior vitreous may be present. Retina can show venous
sheathing in the peripapillary area.
Neuroretinitis: Disk edema associated with macular star (least
common).
On fluorescein angiography (Fig. 15.2B) there is pre-papillary
capillary dilatation and leakage very similar to papilledema. There is
hyperfluorescence of the disk with late leakage possibly involving
the nerve fiber layer. With resolution of the swelling there is a pale
disk with variable loss of the pre-papillary capillaries evidenced on
fluorescein angiogram.
There is ocular pain, which worsens on ocular movements. Age at
presentation ranges between 20-45 years and women are more
commonly affected than men. In children the involvement is bilateral.
Some patients may complain of defective color vision. Patients with
multiple sclerosis may have transient obscurations of vision on exertion
or rise in body temperature (Uhthoffs symptom).
Figs 15.2A and B: Papillitis: (A) color picture (B) FFA
Management
In mild cases (vision 20/30 or better) only observation is indicated,
as the disease is self-limiting.
In cases where vision is 20/40 or worse intravenous (IV)
methylprednisolone 1 gm daily for 3 days followed by oral steroids
1 mg/kg body weight for 11 days is administered.
Optic Neuritis Treatment Trial (ONTT)

Patients treated with intravenous methylprednisolone followed
by oral steroids recovered vision faster than patients treated with
oral steroids alone.
The final visual outcome at 1 year was same with or without IV
steroids.
Patients treated with oral steroids alone had higher rate of
recurrences and increased rate of second eye involvement.
PAPILLEDEMA
Papilledema (Fig. 15.3) is a passive, non-inflammatory, hydrostatic
edema of the optic nerve head, secondary to raised intracranial
pressure (ICP). 1-16 It is usually bilateral, although it may be
asymmetrical. One should differentiate it from papillitis (Table 15.2).
The following are the causes of raised ICP, which in turn cause
papilledema.
1. Intracranial space occupying lesions.
2. Focal or diffuse cerebral edema.
3. Blockage of flow of cerebrospinal fluid (CSF) within the ventricular
system (aqueduct stenosis).
4. Reduced absorption of CSF (meningitis, subarachnoid hemorrhage,
etc).
5. Hypersecretion of CSF by choroid plexus tumor.
6. Increase in CSF viscosity (Guillain-Barre syndrome).
7. Benign intracranial hypertension (pseudotumor cerebri).
Clinical Features
Clinically, papilledema can be classified into four stages.
1. Early stage: In this stage the visual symptoms are absent and visual
acuity remains normal. Ophthalmoscopically the optic nerve head
shows hyperemia, blurring of disk margins. Mild disk swelling
may be present which is best appreciated with slit lamp
biomicroscopy. Absence of spontaneous venous pulsations could
be an early sign of papilledema. Presence of spontaneous venous
pulsations rules out papilledema.
Fig. 15.3: Papilledema

Table 15.2: Differences between papillitis and papilledema

Features Papillitis Papilledema
1. Laterality Unilateral Bilateral
2. Onset Sudden Insidious
3. Loss of vision Sudden Gradual
4. Swelling of the disk Moderate Marked
5. Field defects Central or Concentric
centrocaecal Contraction
scotoma of the visual
field
6. Posterior vitreous Fine opacities Clear
2. Established stage: In this stage patient may be asymptomatic or

complain of transient visual obscuration lasting few seconds. During
these episodes the vision may vary from mild blurring to complete
blindness. Ophthalmoscopically there is gross elevation of optic
nerve head with engorged veins. The disk margins are markedly
blurred and edematous nerve fiber layer obscures the traversing
blood vessels. Peripapillary splinter hemorrhages, cotton wool
spots, choroidal folds and retinal striae (Patons lines) are present.
The macula may have hard exudates forming an incomplete star.
3. Chronic stage: Constriction of peripheral fields may be associated
with the enlargement of the blind spot. Ophthalmoscopically
hemorrhages and disk edema slowly resolves. The cup ultimately
obliterates and nerve fiber layer begins to atrophy giving gray
white color to the disk.
4. Atrophic stage; Secondary optic atrophy ensues. The optic disk is
pale to white with indistinct margins and attenuated and sheathed
vessels in the peripapillary area. Severe atrophy of nerve fiber
layer is present.
Other signs are:
The pupillary reactions are normal.
The color vision is unaffected in early stages but as the chronic
papilledema progresses to optic atrophy the color vision becomes
abnormal.
Visual fieldsIn the initial stages enlargement of the blind spot is
present but as the atrophy sets in constrictions of peripheral fields
are seen.
Unilateral or bilateral sixth nerve palsy may be present due to
stretching of the sixth nerve in the posterior fossa as a result of
raised ICP.
Very rarely papilledema may be unilateral or more pronounced in

one eye than the other (preexisting unilateral optic atrophy, Foster
Kennedy syndrome or unilateral congenital anomaly of optic nerve
sheath) (Fig. 15.4).
On the fluorescein angiogram there is immediate filling of the
dilated capillaries giving hyperfluorescence and leakage from both
the prepapillary and peripapillary capillary plexus and associated
leakage into the surrounding retina.
With resolution of the papilledema the optic disk is flat and pale
and there is lack of filling of the pre-papillary capillary plexus. In
long-standing cases, the prepapillary capillary plexus might
anastomose with the choroidal plexus with a consequent optociliary
shunt vessel. In chronic papilledema the optic disk becomes pale and
there is only minimal leakage of fluorescein.
Ocular symptoms mainly consist of bilateral transient obscurations
of vision lasting few seconds. These are often precipitated by postural
change. Rarely patients may complain of reduced vision. Double
vision, which may be intermittent, can be present in some cases.
Systemic symptoms are associated with raised ICP and consist of
headaches, which are more severe early in the morning or in the
recumbent position. Other symptoms like nausea, vomiting, loss of
consciousness and motor rigidity may be present.
Fig. 15.4: Foster-Kennedy syndrome

Management
The treatment is focused towards the cause of raised ICP. If increased
ICP is related to a mass lesion, removal of the mass is the obvious
treatment of choice. Medical treatment in the form of carbonic
anhydrase inhibitors may help reduce the ICP. If the lesion cannot
be removed, or if the CSF absorption is reduced then treatment is
directed towards shunting of the CSF into the peritoneal cavity
(Lumboperitoneal shunts). In cases of idiopathic intracranial
hypertension optic nerve sheath decompression has been advocated
by some authors to alleviate fluid retention within the surrounding
meninges by creating a small fenestration site within the intraorbital
portion of the nerve. While this procedure has yielded some positive
results, it is extremely complex work and may fail in up to one-third
of all cases.
ARTERITIC ANTERIOR ISCHEMIC OPTIC NEUROPATHY (AAION)
Etiology
AAION is infarction of the prelaminar or laminar portion of optic
nerve head due to inadequate perfusion by posterior cilliary arteries
and is most commonly associated with giant cell arteritis (GCA). GCA
is the most common cause of AAION. Other conditions that may cause
AAION are herpes zoster, rheumatoid arthritis, relapsing
polychondritis, Takayasus arteritis, systemic lupus erythematosus,
and periarteritis nodosa.
Classic Signs
Unilateral visual loss (gross reduction of vision).
Relative afferent pupilliary defect (RAPD) is present in unilateral
cases.
Fundus examination shows pale and swollen disk. Splinter
hemorrhages at and around the disk margins may be present.
Within 1-2 months the disk swelling subsides and optic atrophy
ensues.
Visual fields commonly have altitudinal defect in eyes which can
be tested.
In few cases cranial nerve palsy may be present as an associated
sign.
Systemic signs of GCA are tender, palpable non-pulsatile temporal
arteries.
Involvement of other arteries may lead to myocardial infarction,

renal failure and brainstem stroke. In some cases central retinal
artery occlusion may be an associated finding.
Erythrocyte sedimentation rate (ESR) and C-reactive protein are
invariably raised.
Symptoms
Patients classically present with sudden onset unilateral visual loss
(partial or complete), which may rapidly become bilateral. Average
age of presentation is usually 55 years and above. Women are affected
more commonly than men. Patients may give history of amaurosis
fugax before the onset of visual loss. Diplopia may be present in few
cases. Systemic complaints of headaches, jaw claudication, scalp
tenderness, myalgia, fever and weight loss may be present.
1. Non-arteritic anterior ischemic optic neuropathy
- Patients are younger than those with GCA.
- The visual loss is less severe.
- Systemic hypertension or diabetes mellitus is frequently present.
- Erythrocyte sedimentation rate (ESR) is usually normal.
- Involvement of other eye is less common.
- No benefit from systemic steroids.
2. Optic neuritis (papillitis)
- Affects younger age group (20-40 years).
- The visual loss is severe and recovery is better.
- Pain during ocular movements is frequently present.
- The disk swelling is hyperemic and associated with cells in
posterior vitreous.
3. Compressive optic neuropathy
- Visual loss is gradual in onset and slowly progressive.
- Disk edema may be absent.
- Proptosis or restricted ocular movements are often present
(associated with orbital disease).
- Systemic signs and symptoms of GCA are absent.
Work-up
1. Temporal artery biopsy should be performed in patients where
GCA is suspected before starting steroid therapy or within 2 weeks.
(The biopsy specimen should be at least 2.5 cm long and if the
biopsy is negative but response to steroids is positive biopsy of

opposite artery should be considered).
2. Temporal artery Doppler can also be done.
Risk Factors
In AAION associated with GCA the involvement of other eye without
treatment is seen in 50 percent of cases within days to few weeks.
Management
AAION is a medical emergency and needs to be treated with intra
venous methyl prednisolone 1 gm daily for 3 days followed by 80-
100 mg oral corticosteroids on a slow tapering dose for a period of 6
months to 1 year to prevent involvement of the other eye. Patients of
GCA confirmed by temporal artery biopsy are maintained on initial
dose of corticosteroids for 4 weeks until ESR normalizes and slowly
tapered while monitoring the ESR levels.
Pharmacology
Rare instances of anaphylactoid (e.g., bronchospasm) reactions have
occurred in patients receiving parenteral corticosteroid therapy so
appropriate precautionary measures should be taken prior to
administration, especially when the patient has a history of allergy to
any drug. There are also reports of cardiac arrhythmias and/or
circulatory collapse and/or cardiac arrest following the rapid
administration of large IV doses of methylprednisolone sodium
succinate (greater than 0.5 gram administered over a period of less
than 10 minutes). Bradycardia has been reported during or after the
administration of large doses of methylprednisolone sodium succinate,
and may be unrelated to the speed or duration of infusion.
POSTERIOR ISCHEMIC OPTIC NEUROPATHY (PION)

In the posterior variety (PION) there is no disk edema noted in the
acute phase although the symptoms and clinical findings are otherwise
similar to the anterior variety. The circulation impairment affects the
posterior vessels and there is no impairment of the axoplasmic flow .
The PION is seen more frequently in cases with acute severe blood
loss or sustained hypotension, postsurgical complications, migraine,
collagen vascular diseases and giant cell arteritis.
REFERENCES
1. Hayreh SS. Risk factors in AION. Ophthalmology. 2001;108(10):1717-8.
2. Chan CC, Paine M, ODay J. Steroid management in giant cell arteritis. Br J
Ophthalmol. 2001;85(9):1061-4.
3. Ischemic Optic Neuropathy Decompression Trial: twenty four month update.
Arch Ophtalmol. 2000;118(6):793-8.
4. Beri M et al. Anterior ischemic optic neuropathy. VII. Incidence of bilaterality
and various influencing factors. Ophthalmology. 1997;94(8):1020-8.
5. Jacobs M, Taylor D. The systemic and genetic significance of congenital optic
disc anomalies. Eye. 1991;5(Pt4):470-5. Review.
6. Villalonga Gornes PA, Galan Terraza A, Gil-Gibernau JJ. Ophthalmoscopic
evolution of papilary colobomatous malformations. J Pediatr Ophthalmol
Strabismus 1995;32(1):20-5.
7. Frisen L, Holmegaard L. Spectrum of optic nerve hypoplasia. Br J Ophthalmol.
1978; 62(10:7-15).
8. Traboulsi EI, ONeill JF. The spectrum in the morphology of so called morning
gllory disc anomaly. J Pediatr Ophthalmol Stabismus. 1998;25(2):93-8.
9. Lee MS, Gonzalez C. Unilateral peripapillary myelinated retinal nerve fibers
associated with stabismus, amblyopia, and myopia. Am J Ophthalmol.
1998;125(4):554-6.
10. Principles and Practice of Ophthalmology by Albert Jackobiec, Chapter 20;Page:
2549-60.
11. Atlas of optic neuritis disorders by Thomas J Spur.
12. Focal points 1993, Module 5, Henry JL Van, DyK.
13. Walsh and Hoyts Clinical Neuro-opthalmology. 5th Edition. The Essentials.
Neil R Miller, Nancy J Newman. 196-220.
14. Trobe JD, et al. The impact of optic neuritis treatment trial on the practices of
ophthalmologists and neurologists. Ophthalmology. 1999;106(11):2047-53.
15. Agarwal, et al. Textbook of Ophthalmology 4th vol.; Jaypee; India; 2003.
16. Amar Agarwal. Handbook of Ophthalmology; Slack inc, USA, 2006.
16 Ocular Myopathies
S Soundari
INTRODUCTION
Myasthenia results from the dysfunction of the neuromuscular junction
caused by autoimmunity. Ocular myasthenia most commonly presents
with diplopia, ptosis or both that is variable and characteristically
worse towards the end of the day. Serum antibodies to acetylcholine
receptors are detected in 90 percent of the patients with generalized
myasthenia but only 50 percent will be detected in ocular myasthenia.
Neonatal forms of myasthenia gravis occur in 10 to 15 percent of
children born to the mothers with myasthenia gravis, because of the
placental transfer of antibodies to ach receptor.
The impairment of the neuromuscular conduction causes weakness
and fatigue of the skeletal musculature, but not of cardiac and
involuntary muscles. The disease affects females twice as commonly
as males and may be ocular, bulbar or generalized.
CLINICAL FEATURES
Myasthenic signs and symptoms are variable and tend to worse
with fatigue and stress
Fatigability: When testing for lid fatigue, the patient is asked to
look up without blinking at the examiners hand for 1-2 min. Lid
fatigue on prolonged up gaze is perhaps the most frequently elicited
signs
Peek sign: When the patient is asked to close the lids gently, one or
both inadvertently open slightly or peek
There can absence of Bells phenomenon
Cogans lid twitch: After prolonged down gaze refixation to the
primary position results in overshooting of the upperlid
Hop of the upper lid occurs on looking to the side
Myasthenic ptosis, when unilateral is associated with controlateral

lid retraction
If one eye lid is elevated manually as the patient looks up, the
fellow eyelid will show fine oscillatory movements
Ice pack test: The degree of ptosis improves after the ice pack is
placed on the eyelid for 2 minutes. The test is negative in non
myasthenic ptosis
Diplopia: This is very frequently vertical although any of the muscle
can be involved. Pupil is not involved. A pseudointernuclear
ophthalmoplegia can occur
Saccadic abnormalities like hypometric large saccades, hypermetric
small saccades, quiver movements, hyper fast saccades can occur.
Investigations
Tensilon test: Intravenous injection of edrophonium is the gold standard
for the diagnosis of ocular myasthenia. Edrophonium is a short acting
anticholinestrase which increases the amount of acetylcholine available
at the neuromuscular junction. In myasthenia this results in transient
improvement of symptoms and signs such as weakness, ptosis and
diplopia. Uncommon complications include bradycardia, loss of
consciousness and even death. Lacrimation, salivation and abdominal
cramps are mentioned as common minor side effects. The test should
be done with a resuscitation trolley in hand if in case of sudden
cardiorespiratory arrest.
Objective baseline measurement of ptosis or diplopia with hess
chart should be taken. Intravenous injection of atropine 0.3 mg is
given to minimize muscarinic side effects. Intravenous dose of 0.2 ml
Fig. 16.1: Myasthenia

Ocular Myopathies 199
containing 2 mg of edrophonium hydrochloride is given, if definitive

improvement is noted the test can be terminated.
If no response then the remaining 0.8 ml of 8 mg is injected after
60 seconds if there is no adverse reaction. The response lasts only for
5 minutes.
Perverse reaction like worsening of the strabismus or a paradoxical
response like right hypertropia becoming a left hypertropia after the
injection is considered positive by some.
Neostigmine Test
Intramuscular injection of neostigmine is useful in children. The
effect lasts for 15 minutes to peak and lasts for only 30 minutes
Presence of acetylcholine receptor antibodies is virtually diagnostic
of myasthenia gravis.
Electromyography
Repetitive stimulation and single muscle fiber will show a
decremented response
Sleep test is useful in neonates and babies. There will be
improvement after sleep
Imaging the chest with the computed tomography or magnetic
resonance imaging for the presence of thymoma.
Differential diagnosis for myasthenia gravis:
Isolated or combined third, fourth, sixth or seventh cranial nerve
palsies
Decompensated strabismus
Thyroid disease
Eaton lambert myasthenic syndrome
Botulism
Chronic progressive ophthalmoplegia
Myotonic dystrophy.
Treatment
Treatment of myasthenia gravis can be divided intooptical
treatment, medical, and surgical treatment.
Optical Treatment
Because of the variability of signs and symptoms it is difficult to
treat. For binocular diplopia occlusion of one eye, but it makes the
patient to view monocularly
Fresnel prism can be tried if the ocular deviation is stable for weeks
The crutch glasses are helpful in the case of ptosis.
Medical Treatment
Anticholinergic drugs like pyridostigmine (60 mg) three times a
day. One must be always aware of the cholinergic crisis if too
much of pyridostigmine is given. The patient should be told to
stop if bulbar symptoms or generalized weakness occurs.
Corticosteoids is used along with pyridostigmine. The patient
should be maintained on steroids for months before tapering and
should be a slow tapering for months when the patient is
maintained on low dose of steroids there can relapse or unmasking
of generalized myasthenia.
Immunosuppressant: Azathriopine is effective against myasthenia.
It is given in the dose of 2-3 mg/kg/day
Cyclosporine A, plasmapheresis, mycophenolate and IV gamma
globulin also can be used in generalized myasthenia.
Surgical Treatment
Thymectomy is very effective for ocular myasthenia. The result of
thymectomy for generalized myasthenia are very favorable with
about 35 percent entering complete remission and 50 percent
improving.
Eyelid surgery or ptosis and eye muscle surgery for diplopia are
considered only if it is stable for few months and as a last resort.
CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA

The clinical features are the involvement of the upgaze and then the
lateral movements and may later be affected in all gaze resulting in a
fixed globe. Because the muscle involvement is symmetrical, diplopia
does not usually occur. There is also slowly progressive bilateral ptosis.
Kearns-Sayre Syndrome
This is a mitochondrial cytopathy, inherited from the mother.
It is characterized by pigmentary retinopathy with coarse
granularity.
Conduction defects of the heart can occur. Heart block may result
in sudden death.
Other features are short stature, muscle weakness, cerebellar ataxia,
neurosensory deafness, mental handicap and delayed puberty.
Treatment
Treat the associated conditions. Lubricants for the exposure
keratopathy, base down prisms within reading glasses for reading if
the down gaze is restricted.pacemaker may be required for the cardiac
condition. In ocular pharyngeal dystrophy, for the dysphagia and
aspiration cricopharyngeal surgery. Genetic counseling.
MYOTONIC DYSTROPHY
This is dominantly inherited in the gene of chromosome 19q.
Usually manifest in the third decade. Peripheral muscle
involvement which makes the release of grip difficult which can be
tested with the Hand Shake. A mournful expression caused by bilateral
facial muscle wasting. Slurred speech because of the involvement of
tongue muscles and pharyngeal muscles hypogonadism, frontal
baldness, intellectual detoriation, pulmonary and cardia complication
can occur.
Other ocular features are presenile cataract with polychromatic
luster, ptosis, pigmentary retinopathy, light near dissociation, external
ophthalmoplegia.
ESSENTIAL BLEPHAROSPASM
Blepharospasm can be a very disabling condition in terms of vision
and social life.
More commonly affects female in the older age group. This is a
type of facial dystonia in which there idiopathic tonic contraction of
orbicularis oculi. If it is secondary to any ocular pathology (corneal or
conjuctival foreign body, trichiasis, blepharitis, dry eyes) then it is
called secondary blepharospasm.
Clinical Features
There is a bilateral involuntary lid closure which may be
precipitated by stress, fatigue, social interactions. This is always
bilateral. Disappears during sleep
Secondary ocular changes like ptosis or entropion can occur
This can be differentiated from hemifacial spasm which does not
disappear during sleep.
Treatment: Botulinum toxin given as multiple injections on the upper
and lower lid. The effect generally last for 3 months time. In cases of
secondary blepharospasm treat the underlying cause which is
precipitating the blepharospasm.
Fig. 16.2: Blepharospasm
Other treatment option are medical like benzodiazepine or surgical

like myectomy.
Meiges Syndrome
This is a blepharospasm with midfacial spasm. It may lead on to
compromise of speech , eating and drinking.
Breughel Syndrome
This is associated with severe mandibular and cervical muscle
involvement.
Hemifacial spasm:
This is a tonic clonic spasm of the musculature which occurs even
during sleep. Usually affects the younger age group. It is thought to
be caused by the irritation of the root of seventh cranial nerve by a
compressive lesion. MRI of the cerebellopontine angle should be
obtained to rule out tumor.
Treatment
It includes observation, botulinum toxin injection or neurosurgical
decompression of the seventh nerve (Janetta procedure)
Tourettes Syndrome
This includes multiple compulsive muscle spasms associated with
utterances of bizarre sounds.
Tic Douloureux
Acute episodes of pain in the areas of distribution of the trigeminal
nerve.
Tardive Dyskinesia
This is a orofacial dyskinesia, often with restlessness and dystonic
movements of the trunk and the limbs. Ususlly it is associated with
the long-term use of antipsychotic medication.
Facial Myokymia
Fleeting movements of the facial musculature which may be associated
with stress, multiple sclerosis, caffeine or rarely tumors of the brain
stem.
Lid Apraxia
In lid opening apraxia there is total inhibition of the LPS with no
activation of orbicularis oculi. This results in the lid closure with
difficulty in initiating the lid opening. It is associated with
parkinsons disease, progressive supranuclear palsy, Huntingtons
disease and Wilsons disease.
Lid retraction and poor closure of the lids can occur in Parkinsons,
Parinauds and progressive supranuclear palsy.
BIBLIOGRAPHY
2. American Academy of Ophthalmology- section 5- Neuro-ophthal 2004-2005.
3. Jack J Kanski (6th ed) Clinical ophthalmology, Butterworth Heinmann 2007.
4. Parsons Diseases of The Eye (18th ed) Butterworth-Heinemann International
editions.
Jaypee, India 2003.
17 Miscellaneous
Jeyalakshmi Govindan, S Soundari
PITUITARY TUMORS
INTRODUCTION
Pituitary adenomas occur most frequently between the fourth and
sixth decade of life. Pituitary adenomas with secretory function can
clinically manifest endocrine acivity. It can be either eosinophilic or
basophilic.
Eosinophilic adenomas secretes excessive amount of growth
hormone producing acromegaly in adults and gigantism in the young.
The hands and feet are enlarged, the jaws become prominent, the
tongue is thickened, and the libido fails. Amenorrhea in the female
and impotence in the male develop.
Prolactin secreting adenomas results in galactorrhea and
amenorrhea.
Basophilic adenomas secrete ACTH and produce cushings
syndrome. The clinical features of basophilic adenoma include
adiposity, moon face, hypertension, hypogonadism and osteoporosis.
Visual failure and field defects are less common with functional
adenomas than with nonfunctional adenomas.
Nonsecreting chromophobe adenoma is a highly common
intracranial tumor. Pituitary hypofunction may be present in some
cases.
Headache is the most common neurological manifestation. Bursting
headache is considered as the characteristic of pituitary adenoma.
Loss of vision is the predominant ocular manifestation. The great
majority of the visual symptoms consisted of visual loss in one or
both eyes. The visual loss preceded other symptoms and signs such
as headache and endocrinopathy in many patients.
Miscellaneous 205
The location of the chiasma may be:

Central chiasma: Chiasma lies directly above the sella , normally 80
percent of the individuals have central chiasma.
Post fixed: Chiasma is located more posteriorly on the dorsum
sella,present in 10 percent of the individuals. If there is a involvement
from pituitary tumors the optic nerves are involved first.
Prefixed: Chiasma located anteriorly over the tuberculum sella. If
there is an involvement from the pituitary tumors the tract fibers get
involved first. Present in 10 percent of the normals.
If the chiasma is central both superotemporal fields are initially
affected as inferonasal fibers pass low and anteriorly. As the tumor
grows the inferior temporal field defect progress.
The patient may not present until the central field is involved.
Color desaturation across the midline is the earliest sign of chiasmal
field defect.
Optic atrophy is present in 50 percent of the patients.
The other features which the patient can present with, is the seesaw
nystagmus of Maddox and diplopia as a result of involvement of the
cavernous sign and involvement of cranial nerve.
Investigation
Coronal plane MRI is the investigation of choice
Endocrinological evaluation according to the clinical presentation.
Treatment
Surgery for the pituitary tumor through transsphenoidal approach
Bromocriptine can shrink prolactinomas
Radiotherapy can also be used.
CRANIOPHARYNGIOMA
It is a slow growing tumor of Rathkes pouch. It can interfere with
the hypothalamic function resulting in dwarfism, delayed sexual
development and obesity.
As the tumor compresses from above and behind the infero-
temporal field defects starts early as the upper nasal fibers pass high
and posteriorly.
MRI shows the location of the tumor.
CT scan shows calcification in 60 percent of the cases.
Treatment is mainly surgical. Postoperative radiotherapy may be
helpful but recurrence are common.
HEADACHE
INTRODUCTION
Headache is defined as pain in the head that is located above the eyes
or the ears, behind the head (occipital), or in the back of the upper
neck. Headache is a universal experience as over 90 percent of
individuals have noted at least one headache during their lifetime.
History
The cause can usually be elicited from the history. Points to be noted
are mode of onset, duration, frequency, location, nature of headache,
prodromal symptoms, precipitating relieving factors and medical
history and medication list.
Omnious signs of headache include:
1. Abrupt onset, after 4th decade
2. Progressive symptoms
3. Focal neurological signs
4. Associated with fever, cough, straining
5. Change with position or exertion.
Classification
There are two types of headaches: Primary headaches and secondary
headaches. Primary headaches are not associated with (caused by)
other diseases. Examples of primary headaches are migraine
headaches, tension headaches, and cluster headaches. Secondary
headaches are caused by associated disease. The associated disease
may be minor or serious and life threatening.
Migraine
A migraine is a common type of headache that may occur with
symptoms such as nausea, vomiting, or sensitivity to light. In many
people, a throbbing pain is felt only on one side of the head.
An estimated 18 percent of women and 6 percent of men experience
recurrent headache classified as migraine. It tends to start from age
10 but the peak prevalence is between the ages of 25 and 55.70 to 90
percent of migraine patients have a positive family history. Evidence
suggests involvement of genetic factors in that defective gene encodes
for a voltage dependent calcium channel that ultimately leads to
neuronal excitability.
Miscellaneous 207
Pathophysiology of migraine is not clearly understood. Evidence

suggests the role of trigeminal vascular projections and the
neurochemical serotonin. The hyperexcitability of brain stem neurons
is caused by release of vasoactive neuropeptides which also stimulate
inflammatory cascade dilating meningeal vessels(pain) and activating
portions of cortex(aura).
Variour trigger factors that are thought to bring about migraine
include certain foods, especially chocolate, cheese, nuts, alcohol, stress,
sleep deprivation or birth control pills.
Symptoms and Signs

The headache usually begins in the fronto temporal region, unilateral
but can be bilateral. The onset is usually gradual. The episodes may
start after awakening and are generally relieved by sleep. The aura
of headache precedes by 15-45 minutes. It may include
Scotoma (blind spots)
Fortification (zig-zag patterns)
Scintilla (flashing lights)
Unilateral paresthesia/weakness
Hallucinations
Hemianopia
Classification of Migraine Based on Clinical Presentation

1. Common migraine (80%) - Preceded by nausea, vomiting
(headache without aura) and autonomic symptoms
2. Classic migraine (20%) - Preceded by visual or sensory
(headache with aura) dysfunction
3. Acephalic migraine - Transient neurological events
without headache occur over
the age of 40.
4. Complicated migraine - Migraine with permanent
neurological deficit.
5. Ophthalmoplegic migraine - Ipsilateral palsy of one or more
extraocular muscles as the
migranous headache is resolving.
Common before the age 10.
6. Retinal migraine - Sudden monocular visual loss
without flashes.
Occasionally constriction of
retinal arterioles and venules
noted.
7. Basilar migraine - Bilateral blurring of vision,

field defects, ataxia vertigo,
nystagmus, dysarthria
INTERNATIONAL HEADACHE SOCIETY
Criteria for Migraine Without Aura
A. Five attacks fulfilling criteriaB-D

B. Headache lasting for 4 to 72 hours(untreated)
C. Headache has two of the following:
1. Unilateral
2. Pulsating quality
3. Moderate to severe intensity
4. Aggravated by physical activity.
D. During headache at least one of the following:
1. Nausea or vomiting
2. Photophobia or phonophobia.
Diagnosis
Neuroimaging is rarely indicated in a patient with typical migraine
and a normal examination. CT scan of the head is indicated to rule
out intracranial mass or hemorrhage in selected or atypical cases. MRI
and magnetic resonance angiography are more sensitive. They are
useful if neurologic examination findings are abnormal, the migraine
occurs for the first time after age 40 years, the frequency or intensity
is increasing, and the accompanying symptoms of the attack change.
Lumbar puncture is done when suspecting a diagnosis of subarchnoid
hemorrhage, meningitis or pseudotumor cerebri.
Treatment
Symptomatic or abortive therapy is helpful when the attack occurs

less than twice a week and short lived (Table 17.1). Prophylactic
therapy (Table 17.2) is indicated when the headaches are more frequent
or produce significant disability.Elimination of triggering factors
contributes to prevention.
Miscellaneous 209
Table 17.1: Abortive Therapy in Migraine

Triptans - Sumatriptan, Rizatriptan, Zolmitritan, Eletriptan
NSAIDs - Naproxyn, Indomethacin, ketorolac
Antiemetics - Droperidol, Prochlorperazine, Metoclopramide
Corticosteroids - Prednisone, Methylprednisolone
Miscellaneous - Acetaminophen, caffeine, Ergotamine, Butalbital
Table 17.2: Preventive Therapy in Migraine

Beta blockers - Propranolol, Nadolol
Calcium channel - Verapamil, Nifedipine
Blockers
Antidepressants - Amitriptyline, Fluoxetin
Anticonvulsants - Valproic acid,Topiramate
Miscellaneous - Lithium, Methysergide
Tension Headache
It is the most common type of chronic recurring pain. Tension headache
can be episodic or chronic. The pain is often described as pressure or
tightness. It may occur under emotional stress or intense worry.
Tenderness may be elicited in the scalp or neck.
Criteria for Episodic Tension Headache

A. At least 10 previous headache episodes for filling criteria B-D.
B. Headache lasting for 30 minutes to 7 days.
C. At least two of the following:
1. Pressure/tightening quality
2. Mild or moderate intensity
3. Bilateral location
4. No aggravation with physical activity.
D. Both of the following:
1. No nausea or vomiting
2. Photophobia and phonophobia are absent.
Treatment
The abortive and prophylactic medications for migraine may be tried.
The antidepressants nortryptiline and amitryptiline may be the first
line agents for these patients. Botox injected into the frontal and
occipital muscles has been tried in refractory tension headache.
Cluster Headache
Cluster headache, also known as histamine headache refers to a
grouping of headaches, usually over a period of several weeks. Attacks
usually are severe and unilateral and typically are located at the temple
and periorbital region predominanatly occurs in middle ages
men.Alcohol is common trigger.About 30 to 50 percent of patients
will develop signs of Horner syndrome.
Criteria for Cluster Headache

A. At least five attacks
B. Severe unilateral orbital,supraorbital or temporal pain lasting 15
to 180 minutes
C. Headache associated with at least one of the following:
1. Conjunctival injection
2. Lacrimation
3. Nasal congestion
4. Rhinorrhea
5. Forehead and facial sweating
6. Miosis
7. Ptosis
8. Eyelid edema
D. Frequency of attacks from one to eight per day.
The major entity to exclude in a patient with painful horner
syndrome is carotid dissection.
Treatment
The following may be tried in acute cluster attack:
100 percent oxygen via a facemask
Subcutaneous or intranasal sumatriptan
Intranasal dihydroergotamine
Corticosteroids.
Preventive therapies that are available are lithium, verapamil,
methysergide.
Headache and Ocular Diseases

Headache due to ocular causes are often associated with periorbital
pain and other eye signs. Some of them are given in the table.
Miscellaneous 211
Ocular diseases Examples Clinical features

1. Corneal diseases Corneal erosions Severe pain and foreign
body sensation on awakening
2. Intraocular causes Scleritis, iritis, Angle Pain over the eye, tender to
closure glaucoma palpate and photophobia
is universal
3. Optic nerve Optic neuritis pain on eye movements and
diseases eye may be tender
4. Eye strain Uncorrected Hyperopic Pain over the brow,
Refractive error, spreading to scalp
Convergence insufficiency
The Differential Diagnosis of Headache Disorders
Entity Characteristics of pain

Subarchnoid hemorrage Acute, occipital, worst ever, with meningismus
Brain tumor Subacute dull ache with focal signs
Hypertension Acute frontal or occipital throbbing
Pseudotumor cerebri Subacute, dull, with transient visual loss
and papilledema
Meningitis Acute or chronic, throbbing, with fever
and meningism
Carotid dissection Throbbing pain over periorbital, with horners
syndrome or ipsilateral visual loss
Temporal arteritis Severe pain over the temple, tender
Cough headache Cough induced headache may be associated
with posterior fossa lesions
Hypnic headache Throbbing, global in location after falling asleep,
short lived in elderly
FUNCTIONAL VISUAL LOSS
INTRODUCTION
An apparent loss of visual acuity or visual field with no substantiating
physical signs;often due to natural concern about visual loss combined
with suggestibility and a fear of the worst; best treated with
reassurance.
Functional visual loss (FVL) is frequently encountered in
ophthalmic practice. Identifying such patients is extremely important
in order to avoid unnecessary laboratory testing and secondary gain.
Clinical Presentation
Binocular (rare) and monocular blindness
Decreased visual acuity (LP to 20/30) or fluctuating acuity on
different visit
Visual field defects - monocular hemianopias, bitemporal and
binasal defects, ring scotomas, tubular fields, spiral fields, star-
shaped fields
Diplopia
EOM paralysis
Paralysis, spasm and sluggish accommodation
Voluntary nystagmus
Total color blindness, non-recognition of Ishihara demo plate
Disturbances of reading and writing
Frequent blinking and blepharospasm
Ocular Munchausens syndrome
FVL also has been reported in children due to underlying lack of
parental attention or move to a different school.
Tests for Functional and Simulated Defects

The diagnosis can be made only with high degree of suspicion. The
tests must be objective in nature.
Attitude of the patient: A patient with true vision loss tends to
move cautiously, the hysterical patient move flawlessly; the
malingerer will purposely bump into things.
Visual acuity: This is one of the most important tests as vision
loss is the most common symptom of FVL. Varying test distance is
a useful technique.
Pupillary reflexes: A patient who complains of total blindness with
intact pupillary responses but no cortical lesion is likely to be
functional.
Menace reflex: Blinking to visual threat
Stereopsis: Touching index fingers togther depends on
proprioception and not on vision.
Optokinetic nystagmus: One eye observed ophthalmoscopically,
while the drum revolves before the other. A slight nystagmus will
be easily observed.
Head rotation nystagmus test: Head is rotated passively about
30 alternatively to right and left. The presence of vestibular
nystagmus indicates true blindness.
VEP: The flash evoked visual potential can be used to document
intact visual pathway.
Miscellaneous 213
Tests for Simulation of Uniocular Blindness

Fixation test: Relies on refixation movement. A four diopter base
out prism is placed infront of alleged blind eye. A true blind will
show no refixation movement.
Diplopia test(Graefes test): The suspected eye is covered and
uniocular diplopia elicited in the good eye by bisecting the pupil
with the use of a strong prism.The suspected eye is then quickly
uncovered and simultaneously the prism is slipped over the whole
pupil of the other. If diplopia is still confessed malingering is
proved.
Fogging test: Add progressively strong concave or convex lens in
front of good eye as the patient reads. If reading continues vision
with other eye is proved.
Color tests: Vectographic or duochrome visual acuity testing are
also useful in patients with monocular complaints.
Tests for Simulated Abnormal Visual Field

Monocular visual field defects: The most valuable instrument in
the detection of FVL is the tangent screen as test distance can be
varied. Patients with FVL typically manifest with tubular fields,
i.e. the field remains the same size regardless of test distance.
Also seen are spiraling and crossing isopters.
Binocular field defects: Standard perimetry is performed with
both eyes open patient with FVL never realize the extent of nasal
field of the contralateral eye.But automated perimetry does not
definitely distinguish an organic defect from a functional one.
Central scotomas: It can be tested using the red Amslers grid and
the red/green glasses.If the scotoma disappears when the red lens
is over the affected eye and the green lens is over the good eye
then the patient is functional.
Amblyopia
Optic neuropathies
Retinal degeneration
Cortical visual loss.
When the diagnosis is uncertain MRI with contrast, ERG can be
performed. Rarely neuroimaging with positron emission tomography
may be done for suspected cortical visual loss.
Management
The successful management lies in physicians empathy and
encouragement. Emphasis must be laid on statements such as peripheral
vision seems good and optic nerves are healthy.
Treatment such as prescription of drops, low power spectacles
and retinal test where both eyes are patched and patient is isolated
tend to satisfy them and improve the FVL. Finally psychiatric
consultation is required in some patients.
In children with FVL the prognosis for visual recovery is excellent.
OCULAR MANIFESTATIONS OF
INTRACRANIAL ANEURYSMS
INTRODUCTION
The ophthalmologist may be the first physician to encounter clinical
manifestations of intracranial vascular abnormalities that may herald
devastating neurological complications. The common intracranial
vascular abnormalities are intracranial aneurysms, carotid-cavernous
fistulas and arteriovenous malformations.
Intracranial Aneurysms
An estimated 1 to 6 percent of the general population harbors an
intracranial aneurysm. The annual rupture rate of aneurysms has been
estimated to range from 0.05 to 2 percent, with a higher rate associated
with a previous history of subarachnoid hemorrhage (SAH),
symptomatic clinical presentation (mass effect or cranial neuropathy),
large aneurysm size (>10 mm) or posterior circulation location.
Subarachnoid Hemorrhage
It is the most common manifestation in 90 percent of cases. 10 to 20
percent of patients presenting with aneurysmal subarachnoid
hemorrhage (SAH) will die immediately prior to seeking medical
attention. Others may develop papilledema, subhyaloid hemorrhage
(tersons syndrome), and paresis of lateral rectus of both eyes.
Complications after SAH are rerupture, hydrocephalus, and delayed
cerebral arterial vasospasm with ischemic neurologic deficits.
Miscellaneous 215
Intracranial aneurysm can be congenital, saccular or berry aneurysm

occurring in association with circle of Willis. The ocular manifestations
depend on mechanical pressure on the structures nearby, sudden
increase in size (>1 cm) and rupture.
The unruptured aneurysms of the internal carotid, anterior
communicating, posterior communicating and middle cerebral cause
mass effect. The symptoms can be retro-orbital pain, third cranial
nerve palsy, cavernous sinus syndromes, hydrocephalus, visual field
deficits (Table 17.3), mild to moderate hemiparesis, or hypothalamic-
pituitary dysfunction.
Carotid cavernous aneurysms are uncommon accounting for only
2 percent of all intracranial aneurysms. The aneurysm frequently
involve the abducent nerve early. The pupil sparing third nerve palsy
and ophthalmic(V1) involvement may also occur. The aneurysmal
rupture result in carotid-cavernous fistula (see below) and rarely SAH.
Diagnosis
Transfemoral cerebral angiography is currently the gold standard
for diagnosing intracranial aneurysms. An angiogram provides
important information about site, size, direction of the aneurismal
dome and neck, and relationship with the parent vessel and
perforators. A transcranial Doppler study is useful in detecting the
development of arterial vasospasm.
A CT scan is important for establishing the diagnosis of SAH. An
MRI scan is becoming an important tool for diagnosing a cerebral
aneurysm. Currently, it will detect, with high reliability, aneurysms
that are larger than 5 mm. This ability may be especially useful for
monitoring a patient with a small, unruptured aneurysm. An MRI
scan is also helpful in demonstrating the degree of intramural
thrombus in giant aneurysms. In fact, magnetic resonance angiography
may eventually replace transfemoral cerebral angiography.
Table 17.3: Visual field defects due to intracranial aneurysms

Aneurysms Visual field defects
Internal carotid artery Nasal hemianopia on the affected side and
temporal hemianopia on the other side
bitemporal hemianopia
Anterior and middle cerebral From above bitemporal hemianopia
Posteriorly homonymous hemianopia
Management
Surgical intervention is typically recommended for unruptured
aneurysms (same as CCF below).
Carotid-Cavernous Fistula
Carotid-cavernous fistulas (CCFs) are abnormal communications
between the carotid arterial system and the venous cavernous sinus.
Mechanism of Direct CCF

Trauma (75%): High flow basal skull fracture
Spontaneous causes (25%): Low flow rupture of intracavernous
aneurysm, neurofibromatosis, collagen vascular diseases and
atherosclerotic disease
Ophthalmic consequences of CCF are caused by compression and
ischemia related to increased venous pressure and reduced arterial
pressure.
Clinical signs commonly associated with carotid-cavernous fistula
include proptosis in 94% cases, pulsating exophthalmos (40%), bruit
(75%), fronto-orbital headache, orbital pain (40%), chemosis (71%),
extraocular palsy and diplopia (60%), loss of visual acuity (46%) 5th
cranial nerve involvement in 24.6 percent cases and increased
intraocular pressure and glaucoma.
Causes of Glaucoma in CCF

Elevation of episcleral venous pressure
Elevation of orbital pressure secondary to venous stasis and edema
Secondary neovascular glaucoma
Secondary angle closure from congestion of choroids and forward
shift of lens iris diaphragm.
Indirect CCF or Dural arteriovenous malformations result from
communications between branches of internal or external carotid artery
within the dura of cavernous sinus. They are most often seen in women
over the age of 50 in association with systemic hypertension. Onset is
insidious. The patient may experience diplopia and ophthalmoplegia
(often from CN VI palsy), tinnitus or orbital bruit, and a red, congested
eye that is often mistreated as an ocular infection or inflammation
with arterialized conjunctival vessels and mild proptosis.
Miscellaneous 217
Differential Diagnosis of CCF

Cavernous sinus thrombosis
Retrobulbar hematoma
Unrecognized intraorbital foreign body with cellulitis
Tumor
Diagnosis is accomplished through neuroimaging and arterio-
graphy. Contrast-enhanced CT scan and MRI will demonstrate a
dilated superior ophthalmic vein and cavernous sinus. Ultrasono-
graphy may also demonstrate superior ophthalmic vein engorgement.
Magnetic resonance angiography (MRA) is also very useful in
identifying fistulas as well as particular vessel involvement.
Arteriography is still the gold standard in identifying CCF with vessel
involvement, but due to a small risk of morbidity and mortality
associated with this procedure, we reserve this method for potential
surgical cases (direct rupture of the ICA in high-flow CCF or high-
risk dural CCF).
Management
High-flow CCF resulting from intracavernous rupture of the ICA, in
80-90 percent of cases without treatment result in blindness from
central retinal vein occlusion or glaucoma. Other complications that
may be seen are epistaxis, intracerebral hemorrhage and even death
99 percent treatment done by interventional neuroradiologist by
placement of intravascular coils, carotid artery ligation or finally
surgical clipping.
Indications for Treatment

Glaucoma
Diplopia
Intolerable bruit
Severe proptosis causing exposure keratopathy
Posterior segment ischaemia.
Low-flow dural sinus CCF is very likely to resolve spontaneously
in 20-50 percent of cases. In these cases, periodic observation is the
best therapy. For exposure keratopathy artificial tears are prescribed
and diplopia managed with occlusion therapy. Watch for clinical
deterioration that indicates that a high-risk cortical venous drainage
has developed.
CONCLUSION
Intracranial aneurysm is diagnosed with high index of suspicion that
needs prompt referral to the neurosurgeons to facilitate early
management and therapy.
BIBLIOGRAPHY
1. Amar Agarwal. Handbook of ophthalmology; Slack USA 2005.
2. Bhatti, Tariq M, et al. Delayed Exacerbation of Third Nerve Palsy Due To
Aneurysmal Regrowth After Endovascular Coil Embolization Journal of Neuro-
Ophthalmology. 2004;24(1):3-10.
3. Carotid cavernous fistula Indian journal of otolaryngology and head and neck
surgery 2005;57:65-67.
4. Catalano RA, Simon JA, Krohel GB, et al. Functional visual loss in children.
Ophthalmology 1986;93:385-90.
5. Fahle M, Mohn G. Assessment of visual function in suspected ocular malingering.
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Ophthalmology. 2006;17(6):513-8.
7. Grant T Liu, Nicholas J, Steven L. Galetta Neuro ophthalmology Diagnosis and
management philadelphia, Pennsylvania; W.B. Saunders Company; 2001.
8. Headache classification committee of the international headache society:
Classification and diagnostic criteria for headache disorders, cranial neuralgias
and facial pain. Cephalalgia. 1988-8 (suppl 7):1-96.
9. Hupp SL, Kline LB, Corbett JJ. Visual disturbance of migraines. Surv ophthal
1989;33:221-36.
10. Kline B, Frank J. Bajandas Neuro ophthalmology Review manual: Slack
Incorporated 2004.
11. Lance JW. Current concepts of migraine pathogenesis. Neurology 1993;43
(suppl 1): S11-S15.
12. Miller NR, Keane JR. Walsh and Hoyts clinical neurophthalmology. Baltimore,
Williams and Wilkins, 1998.
13. Peyman-Sanders-Goldberg-third volume-principles and practice of
ophthalmology.
14. Sunita Agarwal, Athiya Agarwal, et al. Textbook of ophthalmology; Jaypee,
India 2003.
15. Walsh and Hoyts clinical neuro ophthalmology Neil miller and Nancy Newman
6th edition 1995 Lippincott Williams.
Examination of a
Neuro-ophthalmology
18 Case
S Soundari
HISTORY
Examination of a neuro-ophthalmology case is crucial.1-4
Onset of the visual loss its progression and the severity whether
associated with pain
Any history of headache, vomiting
Anyaura with the headache
Eye pain: Whether associated with visual loss
Double vision: onset, for distance and near, which direction it is
more
Involuntary movements of the eyeball
Balck outs
Color vision defect
Drooping of the eyelids: Onset, whether it is constant or variable
Any associated double vision, progression and associated features
Any field defects.
Past History
History of medical diseases like diabetes milletus, hypertension
Whether on any drugs for long-term like ATT
Any thyroid eye disease
Myasthenia
Any treatment history like radiotherapy and chemotherapy
Any past CNS problem.
Examination
Best corrected visual acuity:
The processs of examination begins with assessment of visual acuity,
the most common measure of the central visual function is the best
corrected visual acuity which measure the maximal foveal spatial

discrimination, should be obtained with refraction vision should be
tested for both distance and near.
Color Vision by Pseudo-Ishiharas Chart

Testing of color vision compliments assessment of visual acuity. In
optic nerve disease, particularly optic neuritis, the degree of
dyschromatopsia may be proportionality greater than the degree of
smaller visual acuity loss. In macular diseases, acuity and color vision
tend to decline to corresponding degrees. Persistent dyschromatopsia
is common even after recovery of visual acuity in optic neuropathy.
Pseudoischromatic plates are commonly used clinically as a gross test
of color vision because opticneuropathies often manifest prominent
red-green defects.
Pupil Examination
Direct light reflex
Indirect (consensual) light reflex
Swinging flash light test
Near reflex.
DIRECT LIGHT REFLEX

The light reflex is tested in dim light with the patient looking at a
distant target to neutralise the near reflex
The bright light is brought from the side for the right eye. The
pupil will constrict to the bright light briskly
The same is repeated now for the other eye.
INDIRECT LIGHT REFLEX

The eyes are separated by placing a hand in between the right and
the left eye. The patient is asked to fixate at the distant object. The
light is shown to the right eye and the response is seen in the left eye
and the vice versa.
SWINGING FLASH LIGHT TEST

The test is done in dim illumination and the patient focusing at the
distance object. The examiner alternatively illuminates both eyes with
a relatively bright light.
Constant distance, constant duration of the illumination and light
intensity should be maintained so that both eyes must adopt the same
conditions.
Examination of a Neuro-ophthalmology Case 221
When the pupil constricts more slowly and dilates rapidly is called
as relative afferent pupillary defect
NEAR REFLEX
Near reflex is the triad of:
Convergence
Accomodation
Constriction of the pupil.
The near reflex is tested by asking the patient to look at the distance
and to bring the focus to with in 10 cm of the eye.
Confrontation Visual Fields

Make the patient sit opposite to the examiner at 1 meter at the same
eyelevel. Patient should be able to appreciate the object used for
testing the visual fields, for the peripheral visual field.
Each eye has to be tested separately instruct the patient to cover
the left eye and look into the examiners right eye. Present the finger
in each quadrant to test quadrantanopia.
For checking the central field: Red pin is used. The pin is brought
towards the center from each quadrant and the ask the patient to
comment about when he is seeing the pin red. Ask the patient to tell
again when the red becomes faded or disappeared as the pin is moved
inwards. Where the pin has disappeared from that point move the
pin in all directions till it is seen in each direction. This maps out the
blind spot.
For color desaturation two red objects should be presented
simultaneously and compared.
Ocular Movements
Shine the torch light into the patients eye form from front and look
for any obvious tropia. Look for any abnormal head posture or ptosis
Torch light can be used and it should be moved in all nine diagnostic
positions and look for any obvious deviation and nystagmus.
Saccades have to be performed both in the horizontal and vertical
directions. This is performed by showing patient fist and an object
placed for apart. The patient is asked to look from the fist to the
object alternatingly.
Convergence: An object is moved from 50 cm towards the patient.
Normal people will achieve a near point at 10 cm.
If there is any obivious deviation then diplopia charting and hess
charting are done.
Diplopia Charting
In a dark room, a red glass is placed before one eye and a green
before the other to distinguish their images. A bar of light through a
stenopalic slit in a hand torch is then moved about in the field of
binocular fixation at a distance of at least 120 cm from the patient, the
patients head being kept stationery. The positions of the images are
accurately recorded upon a chart with nine squares marked upon it.
The follow data are derived from diplopia charting of:
i. The area of single vision and diplopia.
ii. The distance between the two images in the areas of diplopia.
iii. Whether the images are on the same level or not.
iv. Whethere one image is inclined or both are erect.
v. Where the diplopia is homonymous or crossed.
Test is purely a subjective one. The positions of gaze where the
separation of the images is maximal is found. In that position of gaze,
the furthest displaced image belongs to the eye with the muscle palsy.
Hess Charting
To measure the degree of deviation, especially if torsional and
particularly to measure any progressive increase, the Hess screen is
used.
It consists of a tangent screen marked in lines on a black cloth with
spots at the intersection of 15o and 30o lines with themselves and with
the horizontal and vertical lines. The patient wears a red-green filter
goggles and holds a green light projection pointer. First red in front
of right eye. The examiner holds a red light projection pointer onto
the screen at those spots. The patient is asked to super impose the
green light into the red light. In normal circumstances, the two pointers
should be nearly super-imposed in all nine positions of gaze. The
goggles are then reinversed with red in front of the left eye. The eye
with the green filter is the one, which is tested.
It is useful as a prognostic guide. If the paresis of the muscle
persists, then the shapes of both charts will change as follows:
1. Secondary contracture of the ipsilateral antagonist will develop,
which will show up on the chart as an overaction.
2. This will lead to a secondary (inhibitional) palsy of the antagonist
of the yoke muscle, which will show as underaction.
With further passage of time, the two charts become more and
more concomitant, until it may be impossible to determine which was
the primary pareitic muscle.
Nystagmus
Observe for any abnormal head posture for maintaining in the null
position. Nystagmus to be observed in the primary position. Its plane
like horizontal, vertical, rotatory or see saw to be noted. Its type like
whether it is jerky or pendular.
Its direction, the fast phase of the nystagmus and its amplitude
whether it is fine, medium or coarse.
Ocular motility is then performed and look for dampening of the
amplitude with convergence when the eye accommodates. Cover test
to be performed to identify manifest nystagmus.
Fundus Examination
To look for marginswhether blurred or well defined, its color
whether normal pink color hyperemia or pallor
To look for abnormal vasculature like optociliary shunt
And to look into the macula for the evidence of neuroretinitis.
Visual Fields
Visual loss necessities visual field testing, which aids in localizing the
lesion along the affluent visual pathway and quantifies the defect.
Both kinetic and static and kinetic techniques are important. Testing
may be considered qualitative (looking for the pattern of any visual
field abnormality) or quantitative (measuring the degree of damage).
All points of the equal threshold may be connected to form an
isopter. This contour map represents the outer limits of visibility.
Scotomas are areas of depressed visual function surrounded by
normal visual function. Nerve fibres from the two eyes decussate in
the chiasm. Lesions at the chiasm result in damage to the nasal crossing
fibers and corresponding impairment in the heteronymous temporal
visual fields as Bitemporal hemianopia.
Lesions that injure one optic nerve at its function with the optic
chiasm produce the anterior chiasmal syndrome with functional
scotoma where there is a central visual field loss in one eye accompany
a supero-temporal defect in the opposite eye.
As the fibers course in the retrochiasmal visual pathway (Optic
tract, temporal lobe, parietal lobe and occipital lobe visual radiation).
Crossed nasal fibers from the controlateral eye and uncrossed temporal
fibers from the ipsilateral eye run together. Damage results in
homonymous field defects. Anterior lesions produce incongruous
defects and the posterior damage results in progressively congruous
defects.
Other Cranial Nerve Examination
First Cranial Nerve

Tested by covering each nostril in turn and presenting the patient
with familiar smell like coffee. Irritants must not be used as it
stimulates the trigeminal nerve.
Second Cranial nerve

Visual acuity, pupillary reaction, visual fields, visualizing the optic
nerve head.
Third, Fourth and Sixth Cranial Nerves

Examination of the ocular motility in all nine diagnostic positions.
Fifth Cranial Nerve

Motor part is tested by palpating the temporalis and the muscles
of mastigation when the patient clenches his teeth
Sensory part is tested by checking for light touch in the three
dermatomes corneal reflex to be tested.
Seventh Cranial Nerve

Patient is asked to wrinkle his forehead, smile and blow his mouth.
Attempt to open the eyelids with eyes closed and to look for bells
phenomenon.
Eighth Cranial Nerve

Perform the webers and the rinnes test by using tunning fork.
Ninth and Tenth Cranial Nerves

Gag reflex can be done. Ask the patient to say Ah and to look for any
deviation.
Eleventh Cranial Nerve

Patient should be able to shrug the shoulders against resistance and
to rotate the head against resistance.
Twelfth Cranial Nerve

Make the patient put out his tongue and observe for wasting and
deviation.
CNS Examination
Orientation to time place and person. Memory both short and long
term memory
Tone of the muscle to be checked
Power of the muscle to be graded
Grade 0no movement
Grade 1flicker movement
Grade 2presence of movement when gravity is eliminated
Grade 3movement against gravity
Grade 4movement against resistance but power not full
Grade 5full muscle power.
Reflexes
Both superficial and deep reflexes to be elicited
Biceps, triceps, supinator and finger reflex for the upper limbs
Knee jerk, ankle jerk and babinski reflex for the lower limb.
Coordination
Finger nose test and to look for disdiadokinesia for the upper
limb
Getting the patient to put his heel on the shin and run it up and
down for the lower limb.
Sensation
Pin prick and temperature
Joint and position sense
Vibration sense
Observe the patient for the gait
Spastic gait, wide based gait.
REFERENCES
Jaypee, India 2003.
2. Amar Agarwal. Handbook of ophthalmology; Slack USA 2005.
3. Parsons Diseases of The Eye-(18th ed)-Butterworth-Heinemann International
editions.
4. American Academy of Ophthalmology- section 5- Neuro-ophthal 2004-2005.
Imaging in
19 Neuro-ophthalmology
P Ramesh
COMPUTED TOMOGRAPHY
Principles
Computed tomography (CT) was the first modern imaging technique
which was able to distinguish different soft tissues by measurement
of their different densities. The basis of this technique is the
measurement of different absorption values after exposure to X-rays.
In the slice of interest, the absorption values of parts of a defined
matrix (so-called voxels) are transformed to gray-scale units by specific
algorithms, the reconstruction is shown on a display, and all data are
sampled in a digital manner.
The absorption value is named after its inventor as the Hounsfield
unit (HU) (Hounsfield 1973). It varies linearly in proportion to the
absorption coefficient and is defined arbitrarily: thus, water is defined
at 0 HU, air may have 1000 HU and less, and in bone, values of more
than +1000 HU are measured. The mean values of fat range from 20
to 100 HU, cerebrospinal fluid (CSF) shows about 410 HU, and
brain parenchyma normally presents as 35 HU (white matter) to 45
HU (gray matter).
CT, along with MRI and ultrasound (for orbital pathologies), is
one of the so-called noninvasive imaging modalities. It remains the
method of choice for intracranial emergency screening, also for
suspected fractures, and when an analysis of possible bony changes,
e.g., a calcification is helpful or essential for the decision of the
differential diagnosis. The distinctly different X-ray absorption of
bone, fat, muscles, vitreous body, and lens represents a very good
natural intrinsic contrast of the different orbital tissues.
Imaging in Neuro-ophthalmology 227
Contrast Medium
In normal extracranial parenchymal tissue (e.g., the lacrimal gland)
the effect of diffusion of iodized contrast material out of the lumen of
capillary vessel into the extracellular space is seen as increased den-
sity (>10 HU). As a result of the sum of all contrast medium-filled
capillaries with an intact blood-brain barrier (BBB), the contrast
enhancement of normal brain parenchyma is only 35 HU. The BBB
represents a property of the pial vessel, where the tight junction of
their capillary endothelium prevents a passive diffusion of
macromolecules, such as water-soluble contrast medium (Sage and
Wilson, 1994). In case of a breakdown of the BBB, whether caused by
a tumor or an infection, the continuous endothelial tight junctions are
destroyed, and the extravasation of contrast medium into the
pathologic process leads to a contrast enhancement (> 5 HU).
In CT examination of the orbit, the indication of IV contrast is
limited to suspected vascular lesions, as differential diagnosis is mainly
led by morphological changes. If indicated, two main contraindications
should be considered:
1. A distinct renal impairment may lead to renal failure. The risk of
contrast agent-induced renal failure is high in dehydrated patients,
in those with a known renal or cardiovascular insufficiency, and
in those suffering from plasmocytoma, hypertonus, and
hyperuricemia (Katzberg, 1997). Especially in patients with diabetes
mellitus and an additional renal insufficiency, the risk of contrast-
induced renal failure is about 9 percent (Parfrey et al. 1989).
Although no absolute limiting value can be defined, the serum
creatinine should not exceed >1.5 mg/dl, and the use of nonionic
contrast agent should be standard (Schwab et al. 1989; Uder 1998).
2. In case of a manifest or known history of hyper-thyreosis, an
application of iodized contrast material should be avoided. If
imperatively necessary, it should be applied only after blockage
of the thyroid, in order to avoid a thyrotoxic crisis, still a life-
threatening disease (Kahaly and Beyer 1989). It is recommended
to start prophylactic medication at least 24 hours before the
application and continue it for 14 days, at a dosage of 900 mg
perchlorate per day. In patients at risk, a facultative medication
with 20 mg Thiamazol per day can be administered additionally
(Rendl and Saller 2001).
A known allergic reaction to iodine represents a relative
contraindication, as short-term medication with H1- and H2-blockers
immediately before the exposure to iodized contrast medium can
prevent this complication (Wangemann et al. 1988).
MRI Basic Physical and Technical Principles

of Relaxation, Special Sequences
Magnetic resonance imaging (MRI) is a method to generate cross-
sectional images from the interior of the body based on the physical
phenomena of nuclear magnetic resonance without using ionizing
radiation. Atomic nuclei such as those of 1H, 13C, 14Na, 19F, 23N, and
31P with an odd number of protons and/or neutrons have a magnetic
dipole moment. Hydrogen nuclei are abundant in biological tissue, as

in the hydrogen atoms of water molecules. This is the reason for the
use of hydrogen nuclei in medical MRI. Without the influence of an
external magnetic field, the directions of the innumerable single dipoles
are randomly arranged such that they cancel each other out, resulting
in no macroscopic magnetic dipole moment. However, in the presence
of an external static magnetic field, the small nuclear magnetic dipoles
tend to align in the direction of the field, like a compass needle to the
magnetic field of the earth. The nuclear magnetic dipoles are not
aligned statically, rather they are staggering around the direction of
the external static magnetic field. This phenomenon can be compared
with the tumbling of a top around the direction of the gravitational
force. This movement is called precession. The number of revolutions
of this precession, designated Larmor frequency, depends on the
magnetic moment of the nucleus and the strength of the external
magnetic field applied. In case of a 1.5-T MRI scanner, the Larmor
frequency of the hydrogen nuclei is 63.87 MHz. This characteristic
allows the transfer of energy from an external radiofrequency pulse
to the nuclei provided that the frequency is precisely the same. This
means that there is a resonance between the transmitter and the
macroscopic oscillating magnetic moment, which acts as the receiver.
During energy absorption, the precessing nuclear spin axes circumscribe
a cone that becomes increasingly flat. This can be illustrated as an
exciting nucleus that opens its umbrella. The Brownian motion of
molecules leads to a continuous rearrangement of the dipoles, such
that statistically only one per million (5 ppm: parts per million) of the
hydrogen nuclei are aligned with the direction of an external 1.5-T
field at room temperature. After the termination of the applied
radiofrequency pulse, the macroscopic magnetic field returns to its
prior state by emitting simultaneously decreasing electromagnetic
waves with the precessional frequency. These waves emitted during
the relaxation are measurable and represent values which are attributed
to the brightness of the individual pixels (picture elements) of which
the images are composed by the application of sophisticated
mathematical reconstruction algorithms.
There are two types of relaxation. One is the signal decay of the
sum vector parallel to the strong external magnetic field, which is
termed the longitudinal relaxation or the T1 relaxation. During the
T1 relaxation (spin-lattice relaxation), the excess energy is transferred
from the nuclei to the environment (the term lattice is derived from
crystalline solids and is used here in a broader meaning). The other
relaxation is the signal decay of the sum signal vector perpendicular
to the strong magnetic field and is designated the transverse or T2
relaxation. In T2 relaxation, there is a dispersion of the primarily
synchronized precessional rotation of the spins. One can imagine the
spins as an ensemble of ballet dancers, who initially obey the
instructions of the maestro and start all in the same position (they are
in phase). After this moment, they show a lack of discipline, and each
ballet dancer turns a little faster or slower than the others (loss of
coherence), resulting in a random distribution of the positions (out of
phase). If we return to the spinning direction, at the beginning of this
process we can record the net sum vector of all synchronized (in phase)
individual spins, with a rapid decay as they go off phase. The loss of
coherence is caused by minute local magnetic inhomogeneities around
the macromolecules. As the adjacent spins also exchange excitation
energy with each other, T2 relaxation is termed spin-spin relaxation.
Signals registered from the biological tissue depend on the water or
proton concentration that can be excited and on the relaxation
characteristics. Pure or so-called free water would show a high
concentration of excitable protons and a slow relaxation caused by
only slightly restricted tumbling of small molecules. On the other
hand, protons bound to macromolecules would show a fast relaxation
by dissipating their energy to the environment and a loss of coherence.
The MRI characteristics of tissue are defined by the composition of
these components, represented in this paper in a simplified manner.
Manipulation of the MRI examination parameters enables us to enhance
the differences between the local tissues, resulting in a better inherent
contrast. The terms T1-weighted (T1w), proton density-weighted
(PDw) or T2-weighted (T2w) characterize MRI sequences or images
and define the more pronounced biophysical effect of the specific
image information. Proton density (PD)weighted images are similar
to T2-weighted images, but have a shorter echo of 1050 m and are
less dependent on the relation than on the concentration of protons,
i.e., water concentration in the tissue (Bsiger 1985). The fluid-
attenuated inversion recovery (FLAIR) sequence combines T2-
weighting and suppression of the so-called free, not tissue-bound
water.
After intravenous administration of a MRI-specific contrast

medium, such as gadopentate dimeglumine (biologically inert as
complexly bounded gadolinium, i.e., GD-DTPA or GD-DOTA), a
different take-up by tissues is seen, analogous to the iodized contrast
medium used in CT. The use of contrast medium (in T1-weighted
sequences) can further improve the contrast between anatomical
details and also between normal and pathological tissues because of
its different signal enhancement. If these contrast-enhancing structures
are embedded in primarily hyperintense tissue (such as the extraocular
muscles, or potential lesions within the retro-orbital fat) the signals
will interfere, resulting in a loss of tissue contrast between the
anatomical components. This problem can be solved using a pulse
sequence that suppresses the high signal of the native hyperintense
tissue. In the case of fat, the sequence is designed to be fat-suppressed
(FS). Special MRI protocols enable a differentiation of flowing blood
from nonmoving tissue (so-called stationary tissue), the basis for MR
angiography. In MR angiography, the signal of stationary tissue is
suppressed and the signal of flowing blood is enhanced, without any
application of contrast material.
The so-called diffusion-weighted MRI (DWI) is able to image
molecular diffusion. Tissue-bound water has a restricted molecular
diffusion compared with free water, due to frequent collisions with
macromolecules, in particular proteins. Therefore, tissues with a
different viscosity and a different ratio of intra- and extracellular
spaces show different diffusion properties. For this reason, diffusion-
weighted MRI discriminates reliably an arachnoid cyst filled with
free water and an epidermoid tumor of solid tissue whereas in con-
ventional sequences, liquor and epidermoid tumor can both give the
same signal intensity (Laing et al. 1999; Gizewski 2001). The so-called
anisotropic diffusion of water molecules in the fiber pathways, which
is much more restricted across the fibers than along them, can also be
imaged in different planes (Hajnal et al. 1991). Diffusion-weighted
MRI can disclose an acute infarction at a very early stage, and in the
case of elderly patients with multiple chronic infarctions, it helps to
uncover additional new lesions (Schaefer 2001). It also seems that
diffusion-weighted sequences image a cystic tumor different to the
central colliquation of an abscess, so offering an additional tool in the
differential diagnosis (Kim et al. 1998).
Along with a strong and very homogeneous main magnetic field
and all devices (antennas or coils) to excite the protons by a
radiofrequency pulse and to receive the electromagnetic waves emitted
from them, there is a need for a space-encoding system. Temporary,
superimposed, magnetic gradient fields cause changes of the Larmor

frequency and the phase of spin populations in small volumes (voxels),
with a precise local attribution. Where the magnetic field is stronger,
the precessional frequency is higher, and where the magnetic field is
weaker, the frequency is lower. It is possible to identify the location
of signal generating spin pools by small space-encoded differences of
the frequencies, like distinguishing radio stations. Additional space-
encoded different phases of precessing spin pools are used.
For more superficially located structures, such as the orbits, the
image resolution can be optimized by using phased-array surface coils
instead of the conventional head coil. Surface coils are specially
designed antennas, which can be applied near the region of interest
and fades out disturbing signals from the environment. In case of an
examination of the orbit, they are placed obliquely over both orbits,
in order to lighten the orbital apex. It should be emphasized that a
relatively small unilateral surface coil (with a diameter of about 4 cm)
applied anteriorly over one orbit is only suitable for imaging the
ipsilateral globe and does not provide a more posterior illumination.
Restrictions
Ferromagnetic Material, Pacemaker, Neurostimulator, Ventricular
Shunts with magnetically adjustable valves.
When approaching the temperature of absolute zero, no electrical
resistance as e.g., in the coil of the electric magnet is found. For this
reason, the most frequently used modern high-field MRI scanners
(0.51.5 T) today are based on a superconducting coil of the main
magnet, a system with a liquid helium-cooled main coil.
This strong main magnetic field necessitates a few precautions.
Patients with ferromagnetic implants, e.g., older aneurysm or other
vessel clips, pacemakers, neurostimulators, and traumatically incorpo-
rated metallic-ferromagnetic foreign bodies (e.g. debris arising from
working with metal, or old shell splinters), should not be exposed to
high-field MRI. In addition to the image quality disturbance caused
by the so-called susceptibility artifacts of the ferromagnetic material
(Ldeke et al. 1985) this can endanger the patient (Kanal and Shellock
1993). Whereas metal devices fixed on bone do not present a danger
if exposed to MR, ferromagnetic foreign bodies, or clips in the lung,
abdomen, eye, and adjacent to vessels can twist due to the strong
main magnetic field and lead to a life-threatening complication.
Ventricular shunts with transcutaneous magnetically pressure-
adjustable valves (Medos and Sophy valves) can be maladjusted in
MRI, and therefore the systems have to be checked radiologically
after MRI (Miwa et al. 2001; Ortler et al. 1997). As new magnet-
compatible devices (Wichmann et al. 1997) have only been developed
in the last decade, MRI is still unavailable to most patients with an
implanted pacemaker or neurostimulator. The problems are not only
caused because of the fact that these devices are usually magnetically
programmable, there is an additional risk from the electrodes, which
can act as antennas and interact with the changing electromagnetic
fields. One must be always absolutely certain about the individual
patients magnet compatibility, probably with the result of a rejection
of the patient for MRI if there remains any doubt.
Claustrophobia, Sedation, Surveillance

To perform a MRI examination, it is necessary to bring the entire
patient into the narrow shaft of the equipment, as the optimal
homogeneity of the magnetic field is in the center of the magnet.
Even for an examination of only the head or the orbit, the patient has
to be placed deep inside the MRI. This is mainly a problem for
claustrophobic patients, and thus they need sedation before the MRI
examination. However, a sedated patient placed in this narrow tunnel
is not accessible. In case of deep sedation, special magnet-compatible
monitoring devices are needed for surveillance, including at least
essential peripheral pulse oximetry. In MRI, the acquired data are not
separately sampled sections, as for CT, but the data sampling is
simultaneous for all sections of one sequence and the acquisition time
depends on the examination parameters, e.g., the repetition time
chosen. Therefore, one MRI sequence may last only a few minutes or
even more than 10 min. If the patient moves during this time, a loss of
image quality of all sections results. During MRI of the orbit, the
patient should keep the eyes open and try to maintain a midline resting
position. Consequently, in the case of uncooperative patients, who
are not able to remain motionless, the quality of the images will be
impaired.
Optic Pathway Pathology

congenital pathology/infantile presentations
acquired optic pathway lesions
work-up in systemic diseases
unexpected (incidental) fi ndings
Any neuroimaging procedure should be based on profound clinical
(including ophthalmological if appropriate) examination. This should
allow the neuroradiologist to formulate specific questions.
CONGENITAL PATHOLOGY OF OPTIC PATHWAYS
Infantile Presentations
Micro-ophthalmos/Anophthalmos
Uni- or bilateral micro-ophthalmos/anophthalmos may be seen in
various conditions (Albernaz et al. 1997; Nelson et al. 1991).
Neuroimaging is performed to assess orbital anatomy, optic chiasm,
and posterior visual pathways as well as possible brain malforma-
tions Aicardi syndrome, observed only in girls, is considered to result
from an X-linked mutation that is lethal in boys. The relevant triad
consists of a typical optic disk appearance with chorioretinal lacunae,
infantile spasms, and agenesis of the corpus callosum (Aicardi 1992;
Brodsky et al. 1995). In addition, other central nervous system
malformations are always present, in particular migration anomalies
(heterotopias, polymicrogyria) and midline arachnoid cysts.
Ocular Tumors
Retinoblastoma is the most common intraocular tumor in infancy,
affecting about 1 in 20,000 infants. The most frequent presenting
symptom is leukokoria, also called cats eye reflex. Leukokoria
generally represents an advanced stage of the disease. Computed
tomography (CT) (Fig. 19.1) displays punctate or more homogeneous
areas of calcification in 95 percent of retinoblastomas (Barkovich 1995).
Contrast enhancement of tumor tissue is generally found. Contrast
enhancement is also demonstrable with MRI. T1-weighted images
reveal the tumor as hyperintense, T2-weighted images usually as a
hypointense mass Proton density images may assist in the demarcation
of the tumor. MRI can occasionally provide evidence of distal optic
nerve infiltration. A large proportion of retinoblastomas are genetically
determined, and about a third occurs bilaterally. When tumoros tissue
is also demonstrated in the pineal region by neuroimaging, it is termed
trilateral retinoblastoma. This may already be present on initial
evaluation.
Spasmus Nutans
So-called spasmus nutans typically presents at 612 months with
disconjugate nystagmus, torticollis, and findings have been seen in
girls.
Figs 19.1: CT scan showing trilateral retinoblastoma
Other White Matter Disorders

Apart from PMD, other dysmyelinating conditions can present with
congenital nystagmus. The exact genetic/biochemical basis of these
rare conditions is still unknown.
Septo-optic Dysplasia
Septo-optic dysplasia (SOD) typically presents as congenital
nystagmus. Fundus examination reveals bilateral optic nerve
hypoplasia. In addition, the syndrome consists of an absence of the
septum pellucidum (Hypothalamic-pituitary dysfunction is present in
a minority of patients, presenting as neonatal hypoglycemia and/or
growth retardation (Sorkin et al. 1996). The prognosis is quite variable,
ranging from blindness to useful vision. Affected children may be
mentally retarded. In some children, additional CNS malformations
can be found, in particular hypoplasia of the corpus callosum and
cortical dysplasia (Sener 1996). SOD is unlikely to be a homogeneous
entity. Hypoplasia of the optic nerves and absent septum are also
seen as part of the holoprosencephaly complex (Barkovich 1995). The
septum pellucidum is also mostly missing in rhombencephalosynapsis.
It is suggested that SOD is a vascular disruption sequence (Lubinsky
1997).
Optic Nerve Hypoplasia

Hypoplasia of the optic nerves may be unilateral or bilateral (Figs
19.2 and 19.3). It is not a clinical or pathogenetic entity. We have seen
several children with unilateral optic nerve hypoplasia presenting to
the ophthalmologist with poor vision or strabismus. The intracranial
anterior optic pathways are usually markedly asymmetric, but
additional anomalies are exceptional.
Figs 19.2A and B: (A) Axial, (B) coronal T2-/FSE MRI of a 3-month-old-girl without
visual fixation. Absent septum pellucidum and almost no identifiable optic nerves.
Diagnosis: septo-optic dysplasia
Figs 19.3A and B: Patient clinically blind at 1 year (A) Axial, (B) coronal T2-/FSE
MRI of a 4-year-old boy. Clinically convergent strabismus and bilateral optic nerve
hypoplasia. MRI shows absent septum pellucidum. Diagnosis: septo-optic
dysplasia
Periventricular Leukomalacia
Periventricular leukomalacia (PVL) is a well-known complication of
prematurity before 34 weeks gestational age. PVL affects primarily
the posterior part of the hemispheric white matter. Clinically, it may
go along with spastic diplegia type of cerebral palsy and is often
accompanied by delayed visual development (Jacobson et al. 1996;
Lanzi et al. 1998; Olsen et al. 1997).
MRI allows the detection of specific residual findings: variable
reduction of periventricular white matter predominantly involving
the posterior aspects, increased size of lateral ventricles, often with
an irregular contour (evacuo). The remaining white matter often shows
increased T2 signal, presumably corresponding to gliosis.
Multiple Sclerosis
It is estimated that about 2 percent of patients with multiple sclerosis
(MS) present during childhood. Presenting symptoms may be variable
such as muscular weakness, gait abnormalities, visual symptoms, and
seizures (Ghezzi et al. 1997; Hanefeld 1992). Imaging findings in
pediatric MS are not considered to be different from those in adults
(Barkovich 1995).
Acute Disseminated Encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) or parainfectious
encephalomyelitis is considered an autoimmune response. This
hypothesis is supported by the fact that the gross pathologic and
histologic manifestations resemble those of experimental allergic
encephalitis. ADEM involves primarily white matter but can also affect
cortical and deep gray matter. Children typically develop acute focal
neurologic signs and/or seizures late in the course of a viral illness or
post vaccination). Neuroimaging reveals usually multiple foci of T2
hyperintensities; these may be circumscribed, confluent, or
occasionally affect white matter diffusely (Murthy 1998). Various
patterns of contrast enhancement may be found in the acute/subacute
phase. Differentiation of MS from ADEM is not always possible in
the beginning. The prognosis of ADEM is favorable as a rule, leading
to complete clinical recovery, both from the clinical and the
neuroimaging points of view. Occasionally, sequelae can be found.
Trauma (Nonaccidental Injury)

Injuries to the optic pathways due to head trauma will not be discussed
here. However, we would like to point to so-called nonaccidental
injury by shaking infants less than 6 months of age. The typical

presentation is impaired consciousness and convulsions, often result-
ing in status epilepticus. Typically, bilateral retinal hemorrhages are
present. Extensive cerebral damage in this condition usually results
in permanent neurological sequelae including visual impairment or
even cortical blindness (Ewing-Cobbs et al. 1998). The neuroimaging
correlation in the acute stage is not spectacular, with evidence of brain
swelling and often some interhemispheric blood accumulation. Follow-
up neuroimaging as a rule demonstrates extensive cerebral atrophy.
Neurofibromatosis Type 1
Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder
due to mutations in the very large NF1 gene at chromosome 17q11.
About 50 percent of patients have new germ-line mutations, i.e. they
have no positive family history. The prevalence in most populations
is about 1:4000 individuals. As is evident from the listing of diagnostic
criteria optic pathway glioma (OPG) is such a criterion. OPG are tumors
of infancy; in larger series, the mean age at diagnosis is 45 years
(Figs 19.4A and B).
Diagnostic criteria for neurofibromatosis type 1 The presence of two
or more of the following is diagnostic:
1. Six or more caf-au-lait spots, greater than 5 mm in diameter in
prepubertal children and over 15 mm in post-pubertal individuals.
2. Two or more neurofibromas of any type, or one plexiform neurofi
broma.
3. Axillary and/or inguinal freckling.
4. Optic nerve glioma.
5. A distinctive osseous lesion, such as dysplasia of the sphenoid
wing, thinning of long bone cortex, with or without pseudarthrosis.
6. A first-degree relative (parent, sibling, or offspring) with NF1
according to the above criteria.
OPG are pilocytic astrocytomas. It is important to distinguish
astrocytomas from benign lesions commonly encountered in NF1: T2
hyperintensities are often found in the basal ganglia (particularly
globus pallidus), brainstem, and cerebellum, not enhancing with
contrast and not having space-occupying effects.
Mild proptosis is not uncommon in NF, even in the absence of an
optic nerve glioma. It can be related to sphenoid wing dysplasia, but
often no obvious explanation is evident.
Neurofibromatosis Type 2
Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder
due to mutations at chromosome 22q12. The involvement of the brain
Figs 19.4A and B: (A) Sagittal T1-weighted, MRI of a 26-year-old patient with
NF1. (B) Asymmetrical optic chiasm glioma known for 12 years
structures in NF1 and NF2 are quite different: While NF2 consistently
affects the acoustic/vestibular nerve, this is never encountered in NF1.
NF2 is not associated with optic pathway gliomas.
Diagnostic criteria for neurofibromatosis type 2. The following are
diagnostic:
1. Bilateral vestibular schwannomas; or
2. A first-degree relative with NF2 (Figs 19.5A and B), and either a
unilateral vestibular schwannoma or two of the following: menin-
gioma (Figs 19.6A and B), schwannoma, glioma, neurofibroma,
posterior subcapsular lens opacity, or cerebral calcification; or
3. Two of the following: unilateral vestibular schwannoma multiple
meningiomas either schwannoma, glioma, neurofibroma, posterior
subcapsular lens opacity, or cerebral calcification.
INTRACRANIAL PATHOLOGY OF THE VISUAL PATHWAY
Intrinsic Lesions, Glioma in Adults

The term glioma stands for the corresponding three types of glial
cells. The three major types of gliomas originate from: astrocytoma
oligodendroglioma and ependymoma and the so-called mixed gliomas
that contain two or more different cell types in varying proportions,
most frequently primarily oligoastrocytoma (Okazaki 1989), whereas
intraventricular choroid plexus papilloma and carcinoma are distinct
from ependymoma (Kleihues and Cavanee 2000).
Figs 19.5A to D: T1-weighted, contrast-enhanced MRI of newly diagnosed NF2 in

a 14-year-old girl. MRI shows bilateral acoustic neuromas, meningioma at tip of
left temporal lobe, mass (presumably a meningioma) in suprasellar/left parasellar/
sphenoid area
Figs 19.6A and B: (A) Axial plain CT, (B) axial CT following administration of
contrast medium in a 14-year-old-patient prompted by new onset of diplopia.
Plain CT reveals calcifying right optic nerve sheath meningioma. Following contrast:
left frontal meningioma
Extra-axial Tumors
Due to the fact that extrinsic (or extra-axial) tumors are frequently
benign, the treatment and prognosis are based upon the correct
diagnosis of suspected intracranial extrinsic masses. The use of MRI
is mandatory for these tumors because it has the ability to differentiate
the boundary between the brain parenchyma and the mass itself. The
superior contrast resolution and multiplanar imaging capacity of MRI
enable the identification of anatomic markers as cardinal features of
an extra-axial lesion. Instead of the demonstration of the tissue contrast
of extrinsic masses and brain parenchyma, the definition of boundary
layers between the tumor and the brain surface permits the diagnosis
of an extra-parenchymal intracranial lesion. The boundary layers
represent cerebrospinal fluid (CSF), pial blood vessels, and/or the
dura. CSF clefts are recognized as crescentic bands, frequently only
over a portion of the tumor, with signal intensities similar to those of
spinal fluid: low on T1-weighted, isointense on proton density-
weighted, and high on T2-weighted images. In SE sequences, both
normal anatomic and pathologic vessels are identified as rounded or
curvilinear signal voids at specific locations of the lesion margin. The
use of i.v. contrast agents enables the demonstration of the
compartmentalization of extrinsic lesions, since a large number of
tumors show a specific pattern, including extensive signal enhancement
(meningioma, metastasis), while others show none (epidermoid and
dermoid tumors) (Goldberg et al. 1996).
Metastasis
Intracranial metastasis or secondary brain tumors are defined as
tumors involving the CNS and originate from, but are discontinuous
with, primary systemic neoplasms. They account for 15 to 30 percent
of all intracranial tumors in pathologic series (Okazaki 1989; Nelson
et al. 2000). The most frequent primary malignancies include lung
carcinoma (40% metastasize to the brain), breast carcinoma (roughly
25 percent metastasize to the brain) (Figs 19.7 and 19.8),
hypernephroma, melanoma, and neuroblastoma, the latter occurring
predominantly in children.
All areas of the brain may be affected, with preference for the
corticomedullary junction as the starting point (Okazaki 1989), possibly
due to greater capillarization of this region (Zlch 1986). The sellar
region is the preferred location for hematogenous spread of primary
carcinoma of extracranial origin. In addition to the convexity of the
brain and/or cerebellum, leptomeningeal tumor cells deposit in the
recess of the third ventricle but may also invade the parenchyma of
the hypothalamus and/or chiasm.
Fig. 19.7: A 35-year-old woman with acute vision loss, predominantly of the right
eye, and a history of breast carcinoma. Diagnosis: intra- and suprasellar metastasis.
MRI: Axial T2-weighted FLAIR sequence showing edema of the chiasm and both
optic tracts
Figs 19.8A to D: A 38-year-old-woman with chiasm syndrome, diabetes insipidus,

and a history of breast carcinoma. Diagnosis: hypothalamic and chiasmal
metastasis of breast carcinoma. T1-weighted MRI: (A) Axial native view showing
a slightly hypointense lesion dorsal to the chiasm with apparent invasion.
(B) Corresponding contrast-enhanced view, identifying invasion of the chiasm
and the proximal optic tracts. (C) Coronal native view. (D) Midsagittal contrast-
enhanced view, demonstrating metastatic spread throughout the hypothalamus
and pituitary stalk (D with permission of Mller-Forell 2001)
SELLAR TUMORS
Corresponding to the various tissues, a number of pathologic processes
may occur. More than 30 different pathologic entities, primarily
extrinsic lesions, involving and affecting structures of the sellar and
juxtasellar region have been described (Osborn and Rauschning 1994).
These tumors involve the brain parenchyma secondarily and are often
cured completely without recurrences even if the lesion has reached a
considerable size. Intrinsic brain tumors, which often show a recurrent
clinical course even for benign tumors, develop less frequently in the
sellar region.
Gliomas of the Chiasm

Gliomas of the anterior visual pathway, histologically defined as
pilocytic astrocytoma are uncommon lesions, but account for
approximately 65 percent of intrinsic tumors of the optic nerve. These
lesions most frequently occur in children in the first decade of life
(Dutton 1994), whereas only 10 percent present in patients older than
20 years (Wulc et al. 1989). As most of these gliomas are located in the
intraorbital and intracranial part of the optic nerve (Fig. 19.9),
additional involvement of the chiasm is seen in about 75 percent of
patients However, only 7 percent occur in the chiasm itself, and 46
percent involve both the chiasm and hypothalamus the latter increasing
the mortality rate to over 50 percent, since no specific therapy alters
the final outcome (Dutton 1994).
They are not associated with NF 1 and uniformly show a fatal
course of usually less than 1 year (Rush et al. 1982; Mason and Kandal
1991; Dutton 1994; Hollander et al. 1999).
Imaging Characteristics: MRI as the method of choice relaxation times.
In macroadenomas, MRI enables a high anatomic resolution and
definition of the neighboring tissue, i.e. intracranial optic nerves,
chiasm, and cavernous sinus. In most cases, native, non-contrast-
enhanced images allow accurate and conclusive differentiation of the
tumor and deformed, compressed, flattened visual structures of the
optic of the pre- and post-contrast images does not routinely enable
the definite prediction of tumor invasion of the cavernous sinus. Only
a carotid artery encasement or an extension lateral of the cavernous
sinus towards the temporal lobe is a reliable indicator of cavernous
sinus invasion.
Figs 19.9A to F: MRI: (A) Axial T2-weighted view with a very large, space-occupying,
isointense lesion located in a widened suprasellar cistern, depressing and spreading
the basal vessels, (B) Coronal T1-weighted native view demonstrating pressure
exertion on the widened third ventricle by the central hypointense (necrotic) tumor,
(C) Midsagittal, T1-weighted, contrast-enhanced view with demarcation of the
entire enhancing tumor, compressing and displacing the brainstem, and extending
into the posterior fossa. Note widening of the entrance of the otherwise normally
configured sella (arrow), (D) Coronal native view with intra- and suprasellar lesion
and inferior chiasmal compression. Note the slightly hypointense signal in the
sphenoid sinus, (E) Corresponding contrast-enhanced view with inhomogeneous
contrast enhancement of the intra-/suprasellar, apparently encapsulated lesion,
but homogeneous enhancement in the sphenoid sinus. CORR = Sponding to
sinus inflammation, note the small leptomeningeal enhancement at the base of
the left frontal lobe (arrow), (F) Axial contrast-enhanced view with necrotizing,
encapsulated tumor and leptomeningeal enhancement of the basal frontal sulci
(arrows)
Meningiomas
Meningiomas (Fig. 19.10) associated with hereditary tumor syndrome
such as schwannoma (i.e., in patients with NF2) (Woodruff et al. 2000)
mainly occur in younger patients. Approximately 20 percent of
meningiomas are located in the sellar region, with 50 percent arising
from midline structures such as the sphenoid plane tuberculum sellae
diaphragm sellae, or the dura of the cavernous sinus Globular
meningiomas of the suprasellar or paraclinoid region may produce
early ophthalmological symptoms because of optic nerve.
Figs 19.10A to C: MRI: (A) Axial view in the region of the chiasm, visualizing the
superior region of the right sphenoid wing meningioma and an ipsilateral temporal
meningioma. Note the susceptibility artifacts after left temporal craniotomy.
(B) Coronal view at the cavernous sinus, showing the entire circumference of the
meningioma of the right anterior clinoid process. Note the left temporobasal
parenchyma defect after initial surgery. (C) Coronal view in the region of the
chiasm with tumor extension to the right chiasmal region. Another frontal
meningioma is demarcated in addition to the known temporal meningioma or
chiasmal compression and therefore do not normally exceed 2 cm in diameter
Imaging Characteristics: Due to their high cell density and

psammomatous calcification, meningiomas of the sellar region present
on CT as isodense to hyperdense midline lesions. Diffuse hyperostosis
is particularly apparent on CT images of en plaque meningioma). A
perifocal edema is detected only in the rare cases where the cerebral
cortex is destroyed by this tumor Apart from the differentiation of
pituitary adenomas, where a trans-sphenoidal approach is the
preferred operative procedure, the most important question for
neurosurgeons is the possible invasion of the cavernous sinus and/or
narrowing of the ICA, which is best addressed by MRI.
Craniopharyngioma
They are assumed to arise from Rathkes pouch epithelium and account
for 1.2-4.6 percent of all intracranial tumors and thus represent the
second most frequent tumors of the sellar region after pituitary
adenomas. Craniopharyngiomas (Figs 19.11A to D) show no sex bias
but a bimodal age distribution, with one peak involving children and
adolescents and another one involving adults (Adamson et al. 1990;
Crotty et al. 1995). A clinicopathologic distinction is made between
adamantinous and papillary craniopharyngioma. Most adamantinomas
are hormone-inactive lesions and present as solid tumors with a
variable, at times predominantly cystic component, containing
cholesterol-rich, thick, brownish-yellow fluid with the appearance of
machine oil.
Imaging Characteristics: CT in the axial and coronal views is still justified
for the basic and differential diagnosis of craniopharyngiomas, in view
of the characteristic calcification of parts of the tumor seen in 50-70%
of cases Even in the absence of calcification, the solid tumor parts
appear hyperdense with prominent contrast enhancement, whereas
the cysts seem isodense to CSF and may show enhancement of the
wall MRI enables superior delineation of the tumor extent, especially
on the coronal and sagittal views Morphology and signal patterns are
marked by great variety: adamantinous craniopharyngioma primar-
ily shows a combination of T1-weighted hypoin-tense and T2-weighted
massive hyperintense signal character, (whereas in papillary
craniopharyngioma, a hyperintense signal on T1-weighted and
hypointensities on T2-weighted sequences may dominate. The solid
tumor parts of both types generally show a hyperintense signal on
T1-weighted images and prominent enhancement of the tumor and
cyst wall).
Figs 19.11A to D: (A) Coronal, T1-weighted, contrast-enhanced image with caudal

depression of the third ventricle by the predominantly suprasellar, cystic tumor.
The contrast-enhanced capsule is visualized, while the chiasm is not seen.
(B) Midsagittal view with differentiation of the pituitary stalk (star). Note the
depression, dislocation, and deformation of the brainstem. (C) Axial T2-weighted
image demonstrating the high proton content (high signal) of the oily fluid content
of the cystic tumor region. Note the deformed brainstem. (D) Corresponding T1-
weighted, contrast-enhanced image where a remnant of the chiasm (confirmed
on operation) is seen at the medial right tumor surface (arrow)
Astrocytomas
Astrocytic tumors, represent the most frequent entity of primary brain
tumors with up to 60 percent of all intracranial neoplasms and com-
prise a wide range of age and gender distribution, growth potential,
extent of invasiveness, morphological features, tendency for
progression, and clinical course (Okazaki 1989; Cavanee et al. 2000).
Astrocytomas primarily manifest in adults and may arise at any site
of the CNS, exhibiting a wide range of histopathological features and
biological behavior. Astrocytomas include, e.g. pilocytic and subepen-

dymal giant cell astrocytoma (both WHO I), diffuse low grade
astrocytoma (WHO II), anaplastic astrocytoma (WHO III), and
glioblastoma (WHO IV). These different entities reflect the type and
sequences of genetic alterations acquired during the process of
transformation, where the progression from low grade to anaplastic
astrocytomas and glioblastomas is associated with the cumulative
acquisition of multiple genetic alterations (Cavanee et al. 2000).
Pilocytic Astrocytoma (WHO I)

Pilocytic astrocytomas (WHO I) should be differentiated from diffuse
growing astrocytomas as they are more circumscribed, slow-growing
lesions with different location, morphology, genetic profile, and
clinical behavior.
High Grade Glioma

Diffuse astrocytoma (WHO II) is characterized by a high degree of
cellular differentiation, slow growth, and diffuse infiltration of the
adjacent brain structures. It has a tendency for malignant progression
to anaplastic astrocytoma and, ultimately, glioblastoma). Most of these
patients are adults (presenting with seizures and clinical symptoms,
depending on the localization.
Imaging Characteristic: Although most glial tumors arise from a segment
of only one gyrus (Yasargil 1994), the affected parenchyma (usually
white matter, but an involvement of gray matter is often seen)
demarcates an ill-defined area, hypodense on CT. On MRI,
astrocytomas mostly present as mildly hypointense on T1-weighted
and hyperintense on T2-weighted, due to the fact that they represent
a degenerative microcystic formation, filled with clear fluid, and an
irregular, not always clearly distinguished perifocal edema.
Metastasis
Secondary lesions should always be included in differential diagnostic
considerations of extrinsic and even intrinsic tumors of the sellar
region, particularly with a view to the capacity of some primarily
extracranial tumors to involve the skull base (percontinuitatem) or be
hematogenous. Metastases involving the cavernous sinus
predominantly arise from malignant tumors of the nasal cavity,
growing perineurally or perivascularly via the basal foramina.
Imaging characteristics are nonspecific and similar to those

encountered in more common tumors of the sellar region.
Cavernoma (Syn. Cavernous Hemangioma)

of the Cavernous Sinus
They present as well-defined tumorous lesions with a homogeneous,
intermediate signal on T1-weighted views, a hyperintense signal on
T2-weighted sequences, and homogeneous, massive enhancement
after gadolinium administration.
The Tolosa-Hunt syndrome

The Tolosa-Hunt syndrome (THS) is an inflammatory disease of
unknown origin, limited to the superior orbital fissure and the
cavernous sinus (Smith and Taxdal 1966). The presentation of cranial
nerve paresis of one or several of the cranial nerves passing the
cavernous sinus (N III, N IV, N VI, and N V1) may coincide with the
onset of orbital pain or follow it within a period of up to 2 weeks. The
pain must be relieved within 72 hours after steroid therapy. Although
high resolution CT or MRI can neither exclude nor confirm THS when
a lesion compatible with an inflammatory process is visualized, other
causative lesions as, e.g. a malignant tumor must be excluded.
Clinical and neuroradiological follow-up must be done for at least
2 years, even in patients with negative findings on imaging at onset.
Cystic Lesions
In the differential diagnosis of suprasellar tumorlike lesions, arachnoid
cyst and epidermoid cyst play some important role, along with
Rathkes cleft cyst and hypothalamic hamartoma.
Rathkes cleft cyst, a benign epithelium-lined cyst arising from
remnants of Rathkes pouch, may become symptomatic in the case of
intra-and suprasellar extension, a rather rare condition (Rose et al.
1992). On MRI, signal intensities vary with cyst content from serous
to mucoid (Osborn 1994b).
Arachnoid cysts account for about only 1 percent of all intracranial
space-occupying lesions, but 10 percent arise in the suprasellar region
(Armstrong et al. 1983). Arachnoidal cysts are filled with CSF; the
etiology of these mainly congenital lesions remains poorly under-
stood and controversial, but meningeal mal-development is preferred,
so that minor aberrations of CSF flow through the loose, primitive,
perimedullary mesenchyme are considered to result in a focal splitting
of leptomeninges and the formation of a diverticulum or blind pocket
within the arachnoid membrane (Schachenmayr and Friede 1979;

Becker et al. 1991). As an arachnoid cyst is a well-defined, sharply
demarcated, extra-axial formation filled with CSF, MRI signal char-
acteristics correspond with hypointense signal on T1-weighted and
hyper-intensity on T2-weighted images.
Epidermoid cysts probably arise from inclusion of ectodermal
epithelial elements at the time of neural tube closure (accounting for
0.21% of all primary intracranial tumors, 7 percent of them in the
suprasellar region (Osb or n 1994b). Imaging of these well-delineated,
tumor-like cystic lesions is not always able to differentiate them from
arachnoid cyst, especially since the MRI signal intensities are similar
to CSF in every conventional sequence. DWI confirms the diagnosis.
Multiple Sclerosis
The onset of MS usually occurs in patients aged from 20 to 40 years
(15% before 20 years of age, 10 percent after 50 years) with a female
predominance. Most often, the first and only clinical symptom consists
of impaired vision, presenting as retrobulbar neuritis (RBN), followed
or combined with fluctuating periods of sensomotoric or gait
disturbances. The clinical course of disease progression can be divided
into a relapsing-remitting and a chronic progressive form (Heaton et
al. 1985). For the diagnosis of MS recently published new guidelines
on diagnostic criteria of MS enable the physician to define the diagnosis
for MS, possible MS or nor MS, replacing the diagnostic criteria of
Poser et al from 1983 (McDonald et al. 2001). These guidelines include
the evidence of dissemination in time and space of lesions typical for
MS, objectively determined with clinical and imaging signs. The
obtained imaging criteria for MS should require evidence of at least
three of the four following findings:
1. One gadolinium enhancing lesion or nine T2-hyperintense lesions
if there is no gadolinium enhancing lesion,
2. At least one infratentorial lesion,
3. At least one juxtacortical lesion,
4. At least three periventricular lesions. Additional fi ndings of CSF
abnormalities with the presence of autochtone IgG production
(oligoclonal bands) (McLean et al. 1990), lymphocytic pleocytosis,
and abnormal VEP, typical for MS (delayed but with well preserved
wave form) provide supplement information (Halliday 1993) to
clinical finding of neurological disturbances typical for MS.
Imaging Characteristics: MRI is the imaging tool of choice in suspected
demyelinating disorders (Sartor 1992; Osborn 1994f; vander Knaap
and Valk 1995a; Edwards-Brown and Bonin 1996; McDonald et al.

2001). Although the sensitivity in detecting MS lesions is about 85
percent (Lee et al. 1991), the correlation of neurological symptoms
and localization of imaging findings is generally poor as most foci are
clinically silent (Barkhof et al. 1992), but in some cases a correlation of
clinical and imaging findings is probable The imaging protocol should
include axial and sagittal PD/T2-weighted and FLAIR sequences,
where the demyelinated areas demonstrate a high signal (Filippi et al.
1999a; Reiche et al. 2000). The sagittal view is best in order to show
the characteristic periventricular/ pericallosal, ovoid lesions (so-called
Dawsons finger), caused by the centripetal course of the medullary
veins, representing the perivascular inflammation (Horowitz et al.
1989). T1-weighted native and contrast-enhanced sequences
demonstrate acute or recurrent inflammatory lesions, which normally
enhance contrast media, caused by BBB disruption (Paty 1997; Fazekas
et al. 1999; Reiche et al. 2000). involvement including the optic chiasm
and nerves is the most common site of affection (Mirfakhraee et al.
1986; Okazaki 1989).
Depending on the character of the lesions (solitary, multiple, or
diffuse disseminating), imaging findings may resemble multiple
sclerosis, systemic lupus erythematosus (SLE), non-Hodgkin
lymphoma (NHL) or even inflammatory affections like tuberculous
menengitis) (Edwards-Brown and Bonin 1996; Woitalla et al. 2000).
As periventricular signal intense lesions are seen in about 50 percent
of the patients, the differential diagnosis from multiple sclerosis may
be difficult, but a possible additional leptomeningeal involvement
makes the diagnosis of sarcoidosis more likely (Hayes et al. 1987;
Zajicek et al. 1999; Woitalla et al. 2000). In solitary or multiple lesions
which often demonstrate a contrast enhancement and show a
preference for the diencephalon (the floor of the third ventricle,
hypothalamus) and suprasellar region, solid tumors of the suprasellar
region should be taken into consideration in the differential diagnosis,
along with multiple metastasis, NHL, or Langerhans cell histiocytosis
67 percent of patients with sarcoidosis, leptomeningeal and/or
ependymal involvement is found.
Toxoplasmosis
Corresponding to pathological changes, a target appearance of the
solitary or multiple ringenhancing masses with perifocal edema is
common. Rim or focal nodular enhancement following contrast
administration are seen on CT and also on MRI. The most important,
but sometimes hardly distinguishable differential diagnosis is from

primary CNS lymphoma (Dina 1991). While a periventricular location
and subependymal spread favor lymphoma, more than one lesion,
preferentially adjacent to or in the region of the basal ganglia, make
toxoplasmosis likely (Osborn 1994g).
Acute Disseminated Encephalomyelitis (ADEM)

In contrary to multiple sclerosis, ADEM is characterized by an acute
monophasic disorder, predominantly occurring following a viral
infection or vaccination with a mean latent period of 4-6 days or several
weeks; sometimes it is seen without recognized antecedent. ADEM
may occur at any age, but shows a preference for children or young
adults (Consequently, the simultaneous occurrence of polytopic
neurological symptoms such as hemi-, di- or tetraplegia, cerebellar
ataxia or cranial nerve palsies, combined with optic neuritis and
bladder dysfunction, may lead to the correct diagnosis.
As in all demyelinating diseases, MRI shows best the mainly
subcortical, confluent, bilateral but slightly asymmetric hyperintense
foci on T2-weighted images. Consequently, along with the
monophasic clinical symptoms, if BBB disruption is apparent, a similar
enhancement of the lesion is seen, in contrast to MS, where only acute
foci show a T1 time shortening with signal enhancement.
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Index
A Carotid-cavernous fistula 216

Cavernoma 248
Abducent nerve 132 Cavernous sinus syndrome 121, 126,
clinical features 137
deviation 135 Central Horners syndrome 67
diplopia 135 Cerebellar disorder 30
head posture 135 Cerebellopontine angle tumor 147
ocular movements 135 Chronic progressive external
course 132 ophthalmoplegia 200
cavernous sinus 134 Ciliary ganglion 114, 115
orbit 134 Claudes syndrome 120
superior orbital fissure 134 Cocaine test 67
exit from the brain 132 Coloboma of optic disk 153
lesions 136 Computed tomography 226
nucleus 132 contrast medium 227
Aberrant regeneration of oculomotor principles 226
nerve 121 Conjugated palsies 18
Acute disseminated encephalomyelitis lesions of the basal ganglia
236, 251 overactivity 22
Adrenaline test 67 underactivity 22
Alexanders law 34 lesions of the collicular area 22
Amaurotic pupil 60 lesions of the frontal cortex 18
Anatomy of the supranuclear bilateral underactivity 21
pathways 1 overactivity 18
Anophthalmos 223 unilateral underactivity 20
Aplasia 151 Craniopharyngioma 205, 245
Argyll Robertson pupil 63 Cuneus 83
Astrocytomas 246 Cystic lesions 248
B D
Bells palsy 149 Darkness reflex 59
Benedicts syndrome 119 Diplopia 126, 135
Bergmisters papillae 152 Dissociated palsies 23
Behr syndrome 186 vertical 27
Bielschowskys head tilting test 127 Dolls head phenomenon 50
Blepharospasm 201
Botulinum toxin injection 139 E
Brainstem syndrome 136
Breughel syndrome 202 Enophthalmos 66
Examination of a neuro-
ophthalmology case
C
219
Calcarine sulcus 82 direct light reflex 220
Caloric test 43 examination 219
indirect light reflex 220 Hyperdeviation 126

near reflex 221 Hypoplasia 151
CNS examination 225
confrontation visual I
fields 221
diplopia charting 222 Intermediary tissue of Kuhnt 107
fundus examination 223 International Headache Society 208
Hess charting 222 Internuclear ophthalmoplagia 4, 23
nystagmus 223 classification 24
ocular movements 221 etiology 24
other cranial nerve Intracranial aneurysms 214
examination 224 Intracranial pathology 238
reflexes 225 extra-axial tumors 240
visual fields 223 glioma 238
past history 219 metastasis 240
pupil examination 220 Ischemic optic neuropathy 193
swinging flash light test 220 arteritic anterior
testing of color vision 220 classic signs 193
differential diagnosis 194
F etiology 193
management 195
Facial anhydrosis 67 symptoms 194
Facial myokymia 203 posterior 195
Facial nerve Isolated fourth nerve palsy 126
course 145 Isolated ipsilateral tear deficiency 149
lesions 147 Isolated sixth nerve palsy 138
nucleus 145
Foster Kennedy syndrome 192 J
Fovilles syndrome 137
Functional visual loss 211 Jensen operation 139
Juvenile pilocytic astrocytoma 157
G
K
Geniculate ganglionitis 148
Gliomas 157 Kearns-Sayre syndrome 200
of the chiasm 242 Kjer syndrome 186
high grade 247
Gradenigos syndrome 137 L
Lateral geniculate bodies 79, 87
H
Lesions of visual pathways 93
Headache 206 lateral geniculate body lesions 98
Hemifacial spasm 202 optic nerve type field defect 93
Hess charting 222 arcuate nerve fiber bundle 94
Heterochromia iridis 67 bitemporal hemianopia 96
Hippus 71 central bitemporal hemianopia
Horners syndrome 66 96
Hummelsheims operation 139 junctional scotoma 96
Hutchinsons pupil 65, 120 lower temoporal quantrantic
Hydroxyamphetamine test 67 defects 98
Index 255
nasal nerve fiber bundle Nuclear third nerve paresis 118

defects 95 Nystagmus 32
papillomacular bundle 93 central 51
upper temporal quadrantic cerebellar 51
defects 97 deviational 36
optic radiations and visual cortex hysterical 51
lesion 99 idiopathic congenital 52
occipital lobe lesion 100 jerky 34
parietal lobe lesions 100 neutral zone 34
temporal lobe lesions 99 null zone 34
Lid apraxia 203 nystagmus blockage syndrome 52
Light-near dissociation 64 ocular 35
optokinetic 36-38
M pathological ocular
amaurotic 41
Macular fibers 87 amblyopic 41
Magnetic resonance imaging 228 latent 41
claustrophobia, sedation, Miners 41
surveillance 232 spasmus nutans 41
optic pathway pathology 232 pendular 33
restrictions 231 rotational 48
Malignant gliomas of the optic nerve vertical vestibular 45
169 canal paresis 46
clinical features 169 directional preponderance 47
pathology 170 vestibular 43
prognosis 170 voluntary 51
radiology 170
Marcus gunn pupil 61 O
Meiges syndrome 202
Meningiomas 244 Ocular myasthenia
Metastasis 247 clinical features 197
Meyers loop 81 investigations 198
Micronystagmus 34 electromyography 199
Micro-ophthalmos 233 neostigmine test 198
Migraine 206 tensilon test 198
Millard-Gubler syndrome 136 treatment 199
Miosis 66 medical 200
Morning glory syndrome 155 optical 19
Multiple sclerosis 236, 249 surgical 200
Myelineated nerve fibers 152 Ocular tumors 233
Myotonic dystrophy 201 Oculomotor (third cranial) 109
blood supply 117
N branches 116
cavernous sinus 112
Nasal fibers 86 ciliary ganglion 114
Neurofibromatosis 237 course in superior orbital fissure 112
Non-optic reflex system disorders 30 course in the orbit 114
Nothnagels syndrome 119 exit from the brain 110
Nuclear fascicular syndrome 125 nucleus 109
One and one-half syndrome 26 P

Optic atrophy 185
clinical features 186 Papilledema 190
differential diagnosis 187 clinical features 190
Optic chiasma 75 management 193
Optic disk pit 156 Paradoxical papillary reaction 71
Optic nerve 85, 103 Paralytic pontine exotropia 26
blood supply Parinauds syndrome 22
intracanalicular 107 Partial ipsilateral facial palsy 149
intracranial 107 Periventricular leukomalacia 236
intraocular 108 Petrous apex syndrome 137
intraorbital 108 Pharmacology of the pupil 68
course Phenylephrine test 67
intracanalicular 103 Pilocytic astrocytoma 247
intracranial 103 Pituitary tumors 204
intraocular 104 Polycoria 72
intraorbital 104 Posterior communicating artery
relations aneurysm 120
intracanalicular 104 Pseudo-Ishiharas chart 220
intracranial 104 Pseudo-Argyll Robertson pupil 64
intraocular 105 Pseudo-Gradenigos syndrome 137
intraorbital 104 Pseudo-ophthalmoplegia 17
Optic nerve gliomas 157 Psychosensory reflex 60
association with neurofibromatosis Ptosis 66
160 Pupil abnormalities 71
clinical features 158 Pupil cycle time 60
histopathology 162 Pupil gaze dyskinesis 121
management 167 Pupillary pathways 54
microscopic findings 164 accommodation reflex pathway 57
presenting signs and symptoms convergence near reflex pathway
159 56
radiographic findings 161 papillary dilatation pathway 58
Optic nerve head drusen 152 pupulloconstrictor light reflex
Optic nerve hypoplasia 235 pathway 54
Optic nerve meningiomas 170 Pupil-sparing isolated third nerve
clinical features 171 paresis 121
histopathology 174 Pursuit disorders 29
management 178
radiology 173 R
signs and symptoms 171 Raymonds syndrome 136
Optic neuritis 187 Readers syndrome 68
clinical features 188 Retinoblastoma 233
management 189 Retinochoroidal coloboma 153
optic neuritis treatment trial 189
Optic radiation 81, 88
Optic tract 87
S
Optokinetic nystagmus drum 36 Saccadic disorders 17
Orbital syndrome 121, 126, 138 Secondary optic nerve tumors 179
Index 257
blood-borne metastasis 180 exit from the nucleus 123

extension from adjacent structures orbital course 124
181 superior orbital fissure 124
extension from the eye 179 lesions 125
extension from the meninges and nucleus 123
brain 181
Sellar tumors 242
U
Septo-optic dysplasia 234
Spasmus nutans 233 Uncal herniation syndrome 120
Subarachnoid hemorrhage 214
Subarachnoid space syndrome 126,
137 V
Superior orbital fissure 112, 113, 124
Vergence disorders 29
Supranuclear eye movement systems 2
Vestibular system disorders 30
non-optic reflex system 12
Visual cortex 83, 88
position maintenance system 15
Visual pathways 73
pursuit system 6
blood supply 89
saccadic system 2
lateral geniculate body 92
vergence system 11
optic chiasma 91
Supranuclear lesion 147
optic nerve 90
Swinging flash light test 61, 220
optic radiations 92
optic tract 91
T retina 90
Tardive dyskinesia 203 visual cortex 92
Tersons syndrome 214 lesions 93
Third nerve fascicle syndromes 119 level 73
Tic douloureux 203 lateral geniculate body 79
Tilted disk 155 optic chiasma 75
Tolosa-Hunt syndrome 248 optic nerve 73
Tonic pupil 64 optic radiations 81
Tourettes syndrome 202 optic tract 77
Toxoplasmosis 250 retina 73
Trauma (nonaccidental injury) 236 visual cortex 82
Trigeminal nerve localization 84
exit from the brain 140 lateral geniculate body 87
mandibular division 143 optic chiasma 86
maxillary division 143 optic nerve 85
nucleus 140 optic radiation 88
ophthalmic division 141 optic tract 87
frontal nerve 142 retina 84
lacrimal nerve 142 visual cortex 88
nasocilliary nerve 142
trigeminal cave 140 W
Trochlear nerve 123
course Webers syndrome 120
cavernous sinus 124 Wernickes hemianopic pupil 63
exit from the brain 123 Wolfram syndrome 186

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Manual of

Amar Agarwal MS FRCS FRCOphth

JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD

Amar Agarwal MS FRCS FRCOPHTH

Jeyalakshmi Govindan DO DNB

P Ramesh MBBS DMRD DNB MNAMS FRCR

Reena M Choudhry MD DO DNB FRCS

Saurabh Choudhry MD DO DNB

S Soundari DO DNB FRCS

Neuro-ophthalmology is a complex subspecialty which requires keen

Uday Devgan MD FACS

Understanding Neuro-ophthalmology is a challenge. It took us a long

SUPRANUCLEAR AND INFRANUCLEAR PATHWAYS

Fig. 1.1: Supranuclear pathway

SUPRANUCLEAR EYE MOVEMENT SYSTEMS

frontomesencephalic pathway. When you watch someone watching a game

Fig. 1.2: Horizontal saccade pathway

Fig. 1.3: Vertical saccade pathway

pathways. Disturbances of vertical saccades are much more common

Characteristic of the Saccade

Horizontal Pursuit System Pathway

Fig. 1.4: Horizontal pursuit pathway (slow phase)

Table 1.1: Characteristics of eye movements

One can illustrate this with an optokinetic drum, which is a drum

Parietal Lobe Lesion

Fig. 1.6: Parietal lobe lesion

Vertical Pursuit System

Fig. 1.7: Vertical pursuit pathway

Pursuit or Reflex Vergence

Fig. 1.8: Voluntary vergence pathway

NON-OPTIC REFLEX SYSTEM

Fig. 1.9: Pursuit or reflex vergence pathway

Fig. 1.10: Non-optic reflex system pathway

POSITION MAINTENANCE SYSTEM

eyes are on the ball. Sometimes, there would be an overshooting of

Flow chart 2.1: Supranuclear pathway lesions

In dissociated palsies, there is a misalignment of the eyes as the

Lesions of the Frontal Cortex

Flow chart 2.2: Conjugate palsies

Fig. 2.1: Frontal lobe overactivity: Frontal adversive seizure

Fig. 2.2: Frontal adversive attack

Fig. 2.3: Frontal lobe underactivity

acquired it could be due to multiple sclerosis, Wilsons disease,

Lesions of the Basal Ganglia

Underactivity: Progressive Supranuclear Palsy

Lesions of the Collicular Area

underlying structures in the midbrain and not by damage to specific

Flow chart 2.3: Etiology of inter nuclear ophthalmoplegia

Fig. 2.4: Site of lesions in internuclear ophthalmoplegia

Fig. 2.5: Type I internuclear ophthalmoplegia

Fig. 2.6: Type II internuclear ophthalmoplegia

One and One-Half Syndrome

Fig. 2.7: Type III internuclear ophthalmoplegia

formation (pontine gaze centerPGC) or VI nerve nucleus and

Dissociated Vertical Palsies

Fig. 2.8: One and one-half syndrome- Site of lesion

Fig. 2.9: One and one-half syndrome

conjugate vertical movements are dissociated, one eye being incap-

NON-OPTIC REFLEX SYSTEM DISORDERS

Vestibular System Disorders

Fig. 2.10: Vestibular lesion

Athiya Agarwal, Amar Agarwal

change of stimulus in a gaze system, frequently the vestibular system.

EXAMINATION OF A CASE OF NYSTAGMUS