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Transient
anisocoria,
if recurrent,
in a patient
with a normal
examination is
most often
benign.
ANISOCORIA
Aki Kawasaki
ABSTRACT
Pupillary size and movement are controlled by the autonomic nervous system,
which innervates two muscles of the iris, the radial dilator (sympathetically innervated), and the circular sphincter (parasympathetically innervated). In the normal
state, the distribution of efferent pupillomotor signal to the iris is symmetric between the two eyes so that pupil size is generally equal under varying lighting
conditions. Similarly, reflex pupillary movements, eg, constriction and dilation, are
identical between the two eyes. Asymmetry in the pupillomotor neural output or
their muscular forces results in impaired pupillary movement on one side and
unequal pupil size between the right and left eyes. This chapter reviews the
evaluation of anisocoria, both transient and persistent, in assuming that only one
side (pupil) is faulty and focuses on the neurologic causes of pupillary dysfunction.
Continuum Lifelong Learning Neurol 2009;15(4):218235.
218
TRANSIENT ANISOCORIA
Transient unilateral mydriasis is a clinical sign that often raises the anxiety
level of the consulting clinician because a dilated pupil may herald the
presence of a potentially devastating
neurologic condition. While this statement is true, benign explanations also
exist. In sorting out the different causes
of transient mydriasis, the patients background medical history, mental status,
and ocular motility findings can rapidly
assert whether the situation at hand is
an emergency. The emergency diagnoses to consider and their circumstances
are described in this chapter.
Brain Herniation
In a patient with an intracranial mass
lesion or acute expanding pathology,
such as edema or hemorrhage, unilateral pupillary dilation may indicate
impending transtentorial herniation. In
such a setting, the patient usually has
declining consciousness and develop-
Relationship Disclosure: Dr Kawasaki has received personal compensation for activities with Bayer S.p.A.
Unlabeled Use of Products/Investigational Use Disclosure: Dr Kawasaki discusses the unlabeled use of topical
pilocarpine, topical cocaine, and topical apraclonidine in the clinical evaluation of anisocoria.
KEY POINTS
Cerebral
aneurysm is
not a cause of
isolated,
transient,
unilateral
mydriasis.
Determining
whether
anisocoria is more
notable in bright
light or in dim
light helps to
identify the
abnormal pupil.
Signs and
symptoms of
angle-closure
glaucoma include
acute headache
and unilateral
mydriasis, which
can resemble
serious
intracranial
pathology.
Corneal edema,
conjunctival
injection, and
normal ocular
motility help
distinguish angle
closure from
oculomotor
nerve palsy.
Paroxysmal Discharge
of Irritated Cervical
Sympathetic Nerves
This phenomenon is a rare cause of
episodic unilateral mydriasis described
in occasional patients with injury to
the cervical spine, upper cord, and
brachial plexus. Typically, other signs
Continuum Lifelong Learning Neurol 2009;15(4)
219
" ANISOCORIA
KEY POINT
220
Disorders of the
cervical spine
and cord can
cause transient
mydriasis
and other
intermittent
symptoms related
to paroxysms
of excessive
sympathetic
discharge.
Case 15-1
A 62-year-old retired teacher was referred for recurrent episodes of
headache, visual blur, and mydriasis on the right side. She described
the headache as a dull pain around the right eye, which frequently
appeared at night; the visual disturbance was a fuzziness with haloes
around lights. Symptoms always resolved by the following morning,
and between episodes she felt fine and noticed no visual or pupillary
abnormalities. At the insistence of her husband, the patient sought
medical advice. Her general and neurologic examinations were normal.
Neuroimaging was normal. A tentative diagnosis of subacute angle-closure
glaucoma was made, and following referral to an ophthalmologist, the
diagnosis was confirmed.
Comment. Subacute angle-closure glaucoma causes unilateral eye,
face, or head pain accompanied by blurred vision, typically lasting 30 to
60 minutes. During an attack, the affected eye often becomes red as a
result of conjunctival injection, leading to confusion with cluster headache.
The pupil is poorly reactive, and some patients notice the resulting
anisocoria. The specific historical clues to this ocular diagnosis are the link
to low light (night occurrence with resultant pupil dilation) and seeing
halos (suggesting corneal edema) in the painful eye with mydriasis.
KEY POINT
Benign episodic
mydriasis is a
diagnosis of
exclusion based
on a clinical
description and
a normal
examination
between
episodes.
Case 15-2
A 21-year-old woman recently developed severe and recurring headaches.
A friend pointed out that her pupils were not the same size. When
the patient examined herself in a mirror later that same day, her pupils
appeared isocoric. One week later, the patient noticed that her right
pupil was much larger than her left pupil. Fearing a brain tumor, she
sought urgent medical consultation. Examination revealed no anisocoria;
specifically, her pupils measured 2.5 mm in bright light and 7.0 mm in
darkness, with a brisk pupillary light reflex in each eye. Vision, eyelid
function, and ocular motility were normal. A diagnosis of springing
pupil and tension-type headaches was made.
Comment. Springing pupil, also known as benign episodic mydriasis,
is a diagnosis of exclusion in the patient with transient anisocoria
who has no systemic or neurologic condition known to affect pupils
and who demonstrates no abnormalities on ophthalmic and neurologic
examination.
221
" ANISOCORIA
KEY POINTS
222
In the evaluation
of anisocoria,
remember to
inquire about
any previous
infection,
inflammation,
trauma, or
surgery involving
the eyes,
including laser
procedures.
Look for
irregularity of the
pupillary margin,
distortion of
pupillary shape,
or difference in
iris color as these
suggest a
mechanical
(non-neurologic)
cause of
anisocoria.
FIGURE 15-1
TABLE 15-1
"
Causes of
Anisocoria
Greater in Bright
Light
Mechanical Anisocoria
Ocular trauma
(eg, sphincter tear)
Iridectomy
Angle-closure glaucoma
Iris tumor or mass
"
Neurologic Anisocoria
Oculomotor nerve palsy
Postganglionic
parasympathetic nerve
palsy (eg, tonic pupil)
"
Pharmacologic Anisocoria
Topical anticholinergics
Topical sympathomimetics
Aerosolized bronchodilators
FIGURE 15-3
223
FIGURE 15-2
" ANISOCORIA
KEY POINT
A subtle
oculomotor nerve
palsy might
appear as isolated
anisocoria, but
careful
examination
almost always
reveals
accompanying
motor deficits.
oculomotor nerve. Injury to the oculomotor nerve that damages the pupil
fibers will almost always involve one or
more of the motor fibers as well. Only
with very rare exception is an isolated
pupil abnormality the sole manifestation of an oculomotor nerve palsy.
Thus, in a patient with a large, poorly
reactive pupil, it is important to ask about
diplopia and look very closely for eyelid
and motility deficits (Bartleson et al, 1986)
Case 15-3
224
KEY POINT
For practical
purposes, isolated
unilateral
mydriasis is not
due to an
oculomotor
nerve palsy.
225
FIGURE 15-5
" ANISOCORIA
KEY POINTS
226
The presence of
cholinergic
supersensitivity
is not definitive
evidence of an
Adie pupil but
must be
considered with
the other
clinical findings.
Sectoral palsy of
the iris sphincter
is the earliest
and most
specific feature
of an Adie pupil.
The clinical
features of an
Adie tonic pupil
evolve over time.
When the
pupillary light
reflex is poor,
always check the
near response. If
the near response
is better than the
light response,
then light-near
dissociation
is present.
TABLE 15-2
Time
Course
Day 1
Features
Acute unilateral
mydriasis appears
Pupil does not
constrict to light or to
near effort
Sectoral palsy of iris
sphincter is visible
Week 1
Denervation
supersensitivity
develops
Week 8
Pupil remains
unresponsive to light
Pupil shows a slow
sustained constriction
to near effort
Pupil redilation after
near constriction is
also slow
Week 16
TABLE 15-3
Agent
FIGURE 15-6
227
Test Procedure
Positive Result
Example
" ANISOCORIA
Case 15-4
228
FIGURE 15-7
sometimes on the right side and sometimes on the left side. This has been called
seesaw anisocoria. After instillation of
TABLE 15-4
"
Causes of
Anisocoria
Greater in
Dim Light
Mechanical Anisocoria
"
"
"
Central Lesion
Neurologic Anisocoria
Hypothalamic tumor
Horner syndrome
Brainstem infarct
(eg, Wallenberg syndrome)
Aberrant regeneration of
oculomotor nerve
Demyelinating disease
Pharmacologic Anisocoria
Glaucoma medications
"
Topical anti-inflammatory
"
Common Causes
of Horner
Syndrome
Preganglionic Lesion
Cervicothoracic cord lesion
Physiologic Anisocoria
Paravertebral tumor
Epidural anesthesia
Sympathetic chain tumor
(eg, schwannoma)
"
Postganglionic Lesion
Superior cervical ganglion
tumor (eg, paraganglioma)
Jugular vein trauma
(eg, internal jugular
catheterization)
Parapharyngeal surgery
Internal carotid artery
dissection
Cluster headache
Skull base pathology
(eg, bony fracture,
nasopharyngeal carcinoma)
Cavernous sinus meningioma
Carotid-cavernous fistula
229
" ANISOCORIA
KEY POINT
A mechanically
restricted miotic
pupil does not
dilate well in
darkness or to
topical cocaine
and thus may be
confused for a
Horner syndrome.
However, a
Horner pupil
dilates easily to
direct
sympathomimetic
agonists such as
phenylephrine,
whereas a
mechanically
restricted pupil
remains miotic.
FIGURE 15-8
230
FIGURE 15-9
Agent
Drug Action
Cocaine
(4% or 10%)
Cocaine inhibits
reuptake of
norepinephrine
in postsynaptic
junction
(acts as indirect
sympathomimetic)
Test
Procedure
Put two drops
of cocaine in
each eye; wait
45 minutes
Positive Result
Example
Postcocaine
anisocoria of
1.0 mm or more
(smaller pupil is
Horner pupil)
Before cocaine
231
Apraclonidine
(0.5% or 1%)
Apraclonidines
secondary
property is weak
agonist action at
postsynaptic
1-adrenergic
receptors
(ie, dilute direct
sympathomimetic)
Postapraclonidine
reversal of
anisocoria (larger
pupil is Horner
pupil)
Before apraclonidine
" ANISOCORIA
232
FIGURE 15-10
Reprinted with permission from Liu GT, Volpe NJ, Galetta SL.
Neuro-ophthalmology: diagnosis and management. Philadelphia:
WB Saunders, 2000. Copyright # 2000, Elsevier.
Localization of the lesion using topical 1% hydroxyamphetamine and examination clues helps to direct the
imaging studies. Following instillation
of hydroxyamphetamine, if both pupils dilate, the Horner syndrome is
central or preganglionic; if brainstem
signs are present, then a head MRI with
contrast is sufficient. If brainstem signs
are absent, a head, neck, and chest CT
Case 15-5
A 34-year-old woman developed an acute
left periorbital headache and left ptosis.
Further questioning revealed that she had
spent the day before at an amusement park
with some friends. Examination revealed
a subtle decrease in her left palpebral
fissure due to upper and lower eyelid ptosis
(Figure 15-11). Subtle anisocoria was most
apparent in dim light. Pupillary dilation lag
was present on the left side, and cocaine
testing showed failure of the left pupil to
dilate. A diagnosis of left Horner syndrome
was made, and an emergent MRI revealed
a dissection of the left internal carotid
artery. The patient was hospitalized for
appropriate management.
Comment. An acute and painful Horner
syndrome should be considered to result
from an acute carotid dissection until
proven otherwise (Biousse et al, 1998). Pain
is the most common symptom of carotid
dissection. The location of the pain is usually
a focal area on the face or head ipsilateral
to the side of the dissection. Other sites of
pain include the neck, jaw, pharynx, and
ear. Tinnitus or pulsatile sounds are also
frequent symptoms, and this triad of
findings (pain, Horner syndrome, tinnitus)
is highly suggestive of an acute carotid
dissection. Traumatic dissections are usually
caused by severe blunt head or neck injury,
such as motor vehicle accident, fist fighting,
or manipulative neck therapy. In other
cases, the precipitating event may have
been trivial trauma, including coughing,
sneezing, jerking the head to one side,
painting a ceiling, or riding a roller
coaster, as in this patient.
233
FIGURE 15-11
" ANISOCORIA
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