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Clinical Neuro-Ophthalmology

Surat Tanprawate, MD, MSc(London), FRCP(T)


Neurology Unit, Department of Medicine
Chiang Mai University

The scope of
Neuro-Ophthalmology

Oculomotor system

Visual perception system

conjugate eye movement

Eyelids

Saccadic system

Pupils

Pursuit system

Vergence system

Counter rolling system:


VOR, Ocular fixation
system

Oculomotor
pathway
Supranuclear(UMN)
FEF: horizontal conjugate gaze
Diffuse frontal and occipital:
vertical conjugate gaze

Nuclear (LMN)
Nerve III, IV, VI Nucleus
Internuclear
PPRF, abducen interneuron,
MLF (Horizontal gaze)
riMLF, INC, PC (Vertical gaze)

Infranuclear(LMN)

Fasciculus
Cranial nerve
NMJ
Muscle

Frontal eye fields

Right frontal lobe infarct


Frontal lobe lesion: no diplopia
- Destructive to FEF lesion:

eyes deviate to the lesion


- Destructive to Pontine lesion:

eyes deviate contralateral to the lesion


- Excitatory lesion:

eyes deviate contralateral to the lesion

Dysconjugate eyes

Diplopia (double vision)

pic from wikipedia

Diplopia is the simultaneous


perception of the two images
of a single object that may
be displaced horizontally,
vertically, diagonally

caused by impair EOMs


functions

Diplopia
Monocular
diplopia

Repetitive
images

- Cerebral polyopia
- Non-organic

Binocular
diplopia

Ghosting
image

- Retinal disease
- Refractive error

Misalignment of
the eyes

Nuclear
control

- CN III
- CN IV
- CN VI

Infranuclear
control

- CN palsy
- NMJ disorder
- Muscle disorder

Internuclear
control

Horizontal diplopia
- INO
- PPRF
Vertical diplopia
- INC, riMLF

Infranuclear control

Muscle

Fasciculus
Nerve
NMJ

IO

SR

LR

MR

SO

IO/SR

IR

SR/IO

SO/IR

IR/SO

MR

MR

SR

IO

MR

LR

IR

SO

CONVERGENCE

emedicine.medscape.com/article/

Ophthalmotrope (Ruete, 1857)

bjo.bmj.com/content/93/5.cover-expansion

Nuclear and Internuclear control

III

IV

VI

Nuclear control:
Nucleus III, IV, VI
Horizontal gaze
internuclear control

Vertical gaze
internuclear control

Steps to exam patient with


diplopia
1. Monocular vs Binocular diplopia
2. Exam eye movement: primary position and EOM
3. Other finding
a.eye lids
b.pupils
c.other cranial nerve
4. Specific findings/tests: fatigue test, weakness distribution,
reflex, typical facial features, etc

The action and nerve supply of the extraocular


muscles is demonstrated

Key features
Nuclear and fascicular lesion

Brain stem sign: long tract sign, other CN involvement

Nerve lesion

Neighbourhood sign; other CN, other sign

Internuclear lesion

Specific syndrome; Internuclear Ophthalmoplegia (INO),


WEBINO, One and a half syndrome

NMJ lesion

Fatiguability, not consistent with CN lesion, sign of


myasthenia gravis

Muscle lesion

Not consistent with CN lesion: not consistent with CN lesion,


sign of myopathy

Nuclear and nerve


lesion

CN III

The oculomotor nerve (cranial nerve III)

Feature of CN III palsy

Clinical features: Ophthalmoplegia(MR, SR, IR, IO),


Ptosis, Pupillary dilatation

Part

nuclear complex->fasciculus->basilar>intracavernous->intraorbital

pupillomotor fibres

Stroke syndrome of CN III


palsy
1. Webers syndrome
2. Benedikts syndrome
3. Nothnagels syndrome
4. Claudes syndrome

Webers
syndrome

Contralateral
hemiparesis

Ipsilateral CN III
palsy

+/- contralateral
parkinsonism,
corticobulbar palsy

cause: stroke, mass


lesion

http://www.cram.com

Benedikts syndrome
(paramedian midbrain
syndrome)

Ipsilateral CN III
palsy

Tremor (red nucleus)


http://www.cram.com

Contralateral
extrapyramidal sign

Note: Nothnagel: ipsi CN III palsy+cerebellar ataxia


Claude: Benedikt + Nothnagel

Isolated CN III palsy


Pupils sparing vs non-pupils sparing

Posterior communicating artery aneurysm


causing CN III palsy

Oculomotor nuclear
complex lesion

Key finding of nuclear


complex CN III lesion

incomplete involve muscle innervated with CN III

+/- ptosis

+/- pupillary involvement

+/- other brain stem sign

CN IV

The course of the trochlear nerve in the pons

SO function

Depression

Intorsion

Abduction

SR

IO

IR

SO

Head position in Forth nerve palsy

Head tilt chin down to unaffected side

Forth nerve palsy: which side is affected

Parks three steps to identify


CN IV palsy
1.which eye is higher in primary gaze?
2.Worse in right/left gaze?
3.Which head tilt gives greater hyperdeviation?

Left-Right-Left

Right-Left-Right

Left SO palsy

Right SO palsy

Cause of isolated CN IV in
adult

30% Unknown

20% Ischemic

10% Aneurysm

40% Traumatic

CN IV is the longest and thinnest CN, and passes over the


tentorium cerebelli

CN VI

facial nerve wraps around the nucleus of cranial nerve VI within


the pons

Part: nuclear->fasciculus->basilar
(subarachnoidbase od skull, petrous bone)
>intracavernous->intraorbital

Stroke syndrome related to


CN VI palsy

1. Foville syndrome
2. Millard-Gubler syndrome

Millard-Gubler syndrome (ventral pontine syndrome)

1. CN: ipsilat CN VI + CN VII


2. Corticospinal tr (contralat
hemiparesis)

Cross hemiplegia

Fovilles syndrome (inferior medial pontine syndrome)


1. CN VI, VII (ipsilat.)
2. Corticospinal tr. (hemiparesis)
3. Spinothalamic tr. (contralat.
hemisensory loss)
4. PPRF (lateral gaze weakness)

Basilar portion of CN VI
palsy
1. Acoustic neuroma: hearing
loss+CN VI palsy (first sign is
diminished corneal sensitivity)
2. IICP
3. Nasopharyngeal tumours:
invade the skull
4. Basal skull fracture
5. Gradenigo syndrome: acute
petrositis (CN VI + CN VII
palsy + hearing loss + Pain)
Diagnostic criteria of Gradenigo syndrome
Suppurative otitis media
Pain in the distribution of the trigeminal nerve
Abducens nerve palsy

Bilateral LR could be pseudo sixth nerve


palsy from IICP

Isolated CN VI palsy

Multiple nerve involvement

Cavernous sinus syndrome


Superior orbital fissure syndrome

Cavernous sinus syndrome


Association with
other cranial nerve involvement:
4, 5, 6 CN
oculosympathetic paralysis
Opthalmic branch of trigeminal
nerve

Tend to be partial; alls


muscles innervated are not
equally involved

29

Superior orbital fissure


syndrome

CN 3, 4, 6, V1
30

Superior orbital fissure syndrome


Involve CN 3, 4, 6 and V1 CN 5
distribution +/- oculosympathetic
paresis without anhydrosis
May exopthalmos due to
blockade of the opthalmic veins
Blindness due to extension of
the pathologic process to
involve the optic canal

31

Interneuclear
lesion
Horizontal

Interneuclear ophthalmoplegia (INO): MLF lesion


Bilateral INO : Bilateral MLF lesion
One and a half syndrome: PPRF lesion + MLF lesion

Unilateral MLF lesion


internuclear
ophthalmoplegia
Ipsilateral MR weakness ipsilateral side
Contralat. abducting nystagmus

Interneuclear ophthalmoplegia (INO)

a. Normal primary
position

c. Normal left abduction on


left gaze

b. Left impaired adduction


on right gaze and horizontal
nystagmus of the right eye

d. Normal convergence

Bilateral MLF lesion


Bilateral MLF lesion
Bilateral adducting weakness
Bilateral abducting nystagmus
Impaired vertical vestibular and pursuit
Impaired vertical gaze holding
Gaze evoked nystagmus

Wall eyed bilateral INO : WEBINO


exotropia

One and a half syndrome


Combined lesion :
PPRF and MLF
One and a half
syndrome
Ipsilateral horizontal gaze
palsy
INO

Bilateral PPRF lesion


Bilateral horizontal gaze failure
Sparing vertical gaze
Sparing pupil
May combine with other brain stem sign

Interneuclear lesion
Vertical

Upward and downward gaze failure

Vertical gaze control

Dorsal midbrain syndrome


Parinauds syndrome

A group of eye abnormalities and pupillary dysfunction caused


by lesions of the dorsal midbrain

Clinical syndrome

Upward gaze palsy (supranuclear)

Pseudo-Argyll Robertson pupils: light-near dissociation

Convergence-Retraction nystagmus/ convergence spasm

Eyelid retraction (Colliers sign)

Setting sun sign (conjugate down gaze in primary position)

cases

compression

ischemia/hemorrhage

obstructive hydrocephalus

infection

tumour

Syndrome of
ophthalmoparesis

Miller-Fisher syndrome
Wernicke encephalopathy

Wernickes encephalopathy

Triad

ophthalmoparesis/nystagmus

acute confusion

ataxia

Victor M, et al. The Wernicke-Korsakoff Syndrome and Related Neurologic Disorders Due to
Alcoholism and Malnutrition. 2nd ed. 1989.

Treatment regime

Thiamine IV is recommended

No consensus the dose and duration

IV route

Although standard recommended dose interval is once


daily but half life is 96 mins so may need multiple time
daily

Standard dose is 100 mg iv

EFNS task force: 200 mg three times daily


R Galvin et al. European Journal of Neurology 2010, 17: 14081418

Infranuclear lesion ;
disease of NMJ
disease of ocular muscle

Neuromuscular Junction

Features of NMJ
disorder
Ophthalmoplegia is not consistent with
nerve distribution

Fatigue
Fluctuating course
with other muscle weakness esp. ptosis,
proximal muscle weakness

Eyelid and ptosis

Upper eyelid
Levator palpebral
superioris(CN 3)
Muller
muscle(sympathetic)
Frontalis muscle(CN 7)

Lower eyelid
Capsulopalpebral
fascia(inferior rectus)
Inferior tarsal
muscle(sympathetic)

Ptosis
Non-neurogenic(mechanical)
ptosis

Neurologic ptosis
Congenital ptosis

Uni-bilateral
Partial-complete

Supranuclear
lesion(cerebral
ptosis)
Contralateral
cerebral hemisphere

Pupil involvement
EOM impairment

LMN
Neuropathic(N,
fascicle, CN)
NMJ
Myopathic

Horners
syndrome

Ptosis from Cranial nerve III lesion


- complete or near complete ptosis
- EOM involvement
- Pupil dilatation

Horners syndrome

miosis

ptosis (incomplete, upside down)

anophthalmos

anhidrosis

Afferent visual pathway


and visual loss

Visual loss

Assessment

Visual acuity

Pupillary reflex

Visual field

Fundus

Visual loss
Non-neurological
causes
-refractive error
-corneal problem
-cataract
-glaucoma
-retinal and choroidal
disease

Diplopia
Neurologic causes

Type of visual field


defect
Anterior visual pathway
-Prechiasmatic lesion
-Chiasmatic lesion
-Retrochiasmatic lesion

Posterior visual pathway


-LGB
-Geniculo-occipital lobe
pathway
-Occipital lobe

Assessment causes of visual loss


Unilateral or bilateral
Transient, nonprogressive, progressive
Sudden, gradual onset

Sudden onset
Transient: monocular, binocular
Non-progressive: monocular,
binocular
Progressive : monocular, binocular

Gradual onset

1) Visual loss of sudden onset


Unilateral transient visual loss
Ocular:
angle closure glaucoma,
hyphema, optic disc edema,
partial retinal v. occlusion

Retinal artery:
vasospasm(migraine),
hypoperfusion(hypotension,
hyperviscosity,
hypercoagulable stage),
vasculitis(GCA),
TIA(TMB, amaurosis fugax;
emboli to retinal circulation)

Disc:
trainsient visual
obscuration(chronic swelling
of optic disc)

Optic nerve:
Uhthoffs phenomenon in ON

Visual loss of sudden onset


Bilateral transient visual loss
Disc:
papilledema(transient visual obscuration)

Transient visual cortex dysfunction:


Decrease perfusion: thromboembolism, systemic
hypotension, hyperviscosity, vascular
compression
Epilepsy
Migraine

Visual loss of sudden onset


Non-progressive unilateral sudden visual loss
Hallmark of ischemic of optic nerve or retina
CRAO, CRVO, AION
Central serous choroidopathy
Retinal detachment
Vitreous hemorrhage
Functional visual loss

Visual loss of sudden onset


Non-progressive bilateral sudden visual loss
Occipital lobe infarct
Pituitary apoplexy
Functional visual loss
Head trauma

Visual onset of visual loss


Sudden onset with progressive visual loss
Hallmark of inflammatory lesion: optic neuropathy
Ocular: low-tension glaucoma
Disc: papilledema
Anterior visual pathway:

Inflammation: optic neuritis,


Hereditary: LHON
Toxic neutritional optic neuropathy
Compression: aneurysm, tumor, dysthyroid optic neuropathy
Radiation
Paraneoplastic retinopathy/optic neuropathy

2) Visual loss of gradual onset


Hallmark of compressive lesion
Affect: prechiasmal, chiasmatic visual pathway
Common: pituitary tumor, aneurysm, craniopharyngioma,
meningioma, glioma
Granulomatous involvement: TB, sarcoidosis
Ocular dysthyroidism
Hereditary or degenerative of retina or optic nerve
Normal tension glaucoma
Chronic papilledema from pseudotumor cerebri
Medication: toxic to optic nerve
Radiation damage to anterior visual pathway
Rapid pregressive paraneoplastic retinopathy/optic neuropathy

Pupillary and eyelid


abnormality

Pupillary abnormality
Evaluation of pupillary abnormality
Size and shape of pupil
Reaction of pupil

Light reaction
Direct light reflex
Indirect(consensual) light reflex:
swing flash light test
Near(Accommodation) reflex:

92

Abnormal pupil
Size abnormality

Abnormal pupillary
reflex
Unequal size(anisocoria)
Abnormal equal size:
miosis VS mydriasis

Shape abnormality
Pupillary irregularity

Abnormal light reflex


Abnormal direct light reflex
Abnormal consensual light
reflex: RAPD

Abnormal near reflex


Light- near dissociation

Anisocoria
Opthalmologic
anisocoria

Simple(physiologic
) anisocoria
Neurologic anisocoria

Symp: ptosis,
anhydrosis
Parasymp: ptosis.
EOM

Pupil dilatation pathway


abnormality
(anisocoria greater in
darkness)
-Horners syndrome
-old Adies(Tonic) pupil
-Aberrent degeneration

Visual
system

Pupil constriction
pathway abnormality
-Adies pupil
-CN3 palsy

Poorly reactive pupil without


anosocoria
Large pupil
Hypothalmic lesion, midbrain lesion, syphilis,
botulism, MFS, autonomic neuropathy, drug/toxic,
anxiety

Small pupil
Old age, syphilis, diabetes, long standing Holme
Adies pupil, congenital, drug/toxic

Common pupil syndrome

Adie Tonic pupil


Adies syndrome
Light near dissociation
Argyll-Robertson pupil
Parinauds syndrome
Horners syndrome

Tonic (Holme-Adie)pupil
Typically: unilateral mydriasis in healthy
young women
Acute: large
Months to years: small
React to light: sluggish or slow reaction to
light and slow(tonic) near response
Cause: postganglionic parasympathetic
denervation

Light near dissociation


Absent or impair light reflex with preserved
accommodation reflex and convergence
Cause:
DM: small vv disease
Dorsal midbrain syndrome
Argyll-Robertson pupil
Adie pupil
Afferent visual pathway lesion

Argyll-Robertson pupil

Small, irregular, unequal


Normal afferent visual system
Light near dissociation
Cause: neurosyphilis

Relative afferent pupillary defect


Marcus Gunn pupil

Swing flashlight test


Cause
Asymmetrical optic nerve disease
Extensive retinal damage

Pupillary irregularity

Most: local disease of iris


Syphilis
Ischemia
Posterior synechiae
Traumatic iridoplegia
Degenerative disease of iris
Holmes Adie syndrome

Localization of Horners
syndrome

Nystagmus

Nystagmus

Ancient Greek (nustagmos (Ancient


Greek,"nodding, be sleepy")

Involuntary biphasic rhythmic ocular oscillation in


which one or both phase are slow

The slow phase is responsible for the initiation


and generation of the nystagmus, whereas the
fast (saccadic) phase i a corrective movement
bringing the fovea back on target

Type: jerk (direction to fast phase) ; pendular


nystagmus

Mechanism
Nystagmus may result from dysfunction

of the vestibular ending organ,


vestibular nerve, brainstem, cerebellum,
or cerebral centre for ocular pursuit

Peripheral vs Central nystagmus


Peripheral nystagmus

None or mild vertigo

Often chronic

May be purely vertical or


torsional

Visual fixation usually has no


effect

Downbeat, upbeat, torsional

Etiologies commonly vascular,


demyelination, pharmacologic,
toxic

Severe vertigo
Minute to Day to weeks duration

Hearing loss, tinnitus associated

Usually horizontal with torsion

Very rarely purely vertical or


torsional

Central nystagmus

Commonly peripheral vestibular


organ dysfunction: labyrynthitis,
menieres disease

A schematic illustration of nystagmus waveforms

(A) pendular nystagmus

(B) an accelerating velocity


exponential slow phase jerk
nystagmus (CN)
(C) a decelerating exponential
slow phase jerk nystagmus
(MLN)
(D) a linear or constant velocity
slow phase jerk nystagmus
(MLN)
In (A) a slow phase is followed by a slow phase while in (B)(D)
a slow phase is followed by a fast phase

Mechanism

Pendular nystagmus: is central (brainstem/


cerebellum)

Jerk nystagmus:

linear (constant velocity) slow phase: peripheral


vestibular dysfunction

slow phase has decreasing velocity exponential:


brainstem neural integrator, cerebellar

slow phase has increasing velocity exponential:


central in origin (usual form of congenital
nystagmus)

The Neurologist
CMU
The Neurologist
CMU

Cocaine prevents the reuptake of norepinephrine at the motor endplate


and thus prolongs its action on the effector cell.

Differentiation between physiological anisocoria and


Horner's syndrome