Neuro-ophthalmologic Disorders

Clinical evaluation of NO patients

Optic neuritis
 Papilloedema
 Optic nerve atrophy
 Disorders of cranial nerves
 Nystagmus
Pupillary abnormalities
Objectives
To take comprehensive history and perform
evaluation of patients with neuro-ophthalmic
disorders
Develop knowledge and skills on neuro-ophthalmic
patient diagnosis, formulate DDx & order appropriate
investigation or tests.
Describe the clinical presentation of common
neuroophthalmic disorders and able to provide first
line treatment.
Able to identify and refer patients to the
ophthalmologist/neuro-ophthalmologist for further
evaluation and management
Clinical evaluation
History
 Identification: Name, age, sex, occupation
 type of involvement (unilateral Vs bilateral)
unilateral ….anterior to optic chaism
Bilateral ….bilateral retinal, optic nerve ,chiasmal or
retrochaisamal
 Time course of visual loss
sudden in min…ischemic often embolic
rapid over hrs …ischemic character of optic nerve
days to weeks …ischemic inflammation
months to years….compressive
gradual…….toxic lesions
Associated symptoms
pain epsilatral to visual loss, on eye mov”t, globe
tenderness…..optic neuritis
facial numbness & diplopia…orbital or cavernous sinus
lesion
headache ….intracranial mass or giant cell artrities
Medications & allergies
Medical history
Functional inquiry
Family history and social history
Examination
aim…detect, quantify & localize site of loss and
determine the etiology
1) Best corrected visual acuity
with pinhole
refract for near and distance
if done correctly near vision= distance
near vision> distance…. macular dx, nuclear cat
distance> near …polar or posterior capsular cat
Look for eccentric fixation…central scotoma
one side chart reading
If patient reads single optotype better than whole line …
ambylopia
 2.Pupils
Record the size , difference in size (Anisocoria)
Anisocoria greater in the dark suggests sympathetic disease,
such as Horner syndrome.
Anisocoria greater in the light suggests parasympathetic
disease, such as Adie tonic pupil
 Pupillary light reflex
RAPD (or Marcus Gunn pupil)
hallmark of impaired optic nerve conduction
Reliable and sensitive indicator of asymmeric optic nerve
function
RAPD may be seen in severe macular and retinal dx
 Near response
3. fundus examination
focus on clarity of view and apperance of structures.
4. Visual field
5. Color vision … macular diseases result
in corresponding decline of color vision and visual
acuity test
Optic nerve dx have greater color vision loss than
visual acuity
External examination
Ocular motility
Anomalous head positions
Patients with CN IV palsy usually tilt their heads to the
side opposite the palsy.
 Patients with CN VI palsy usually turn their heads
toward the side ipsilateral to the palsy to avoid
diplopia.
Chin-up positions may be seen with ptosis
Eyelids
Lid retraction
Ptosis
Orbit
Proptosis
CNs V, VII, and VIII
Extra ocular examination
BP in papilledema
Neurologic exam
 Optic neuritis
 is inflammation of the optic nerve
 usually occurs in young patients (mean age 32yrs),
 most often female (77%) and presents as sub acute monocular visual
loss developing over days to weeks.
 Clinical presentation.
 Reduced VA
 Periorbital pain , particularly with eye movement in 92% 0f cases.
 Dyschromatopsia, particularly for red and is often more sever than is
loss of visual acuity
RAPD is present unless the optic neuropathy is bilateral and symetric.
 central visual field loss.
There are two form of optic neuritis
1. Anterior(papillitis)
Accounts for 35%of cases of optic neuritis
The optic disc is diffusely edematous and hyperemic
 Is more common in post viral and infectious neuritis.
 Children in particular manifests post viral neuritis which is bilateral
and is associated with profound visual loss.
2. Retro bulbar neuritis
accounts for 65%of cases and is associated with normal optic disc
appearance.
It may be isolated or associated with demyelinating disease(like multiple
sclerosis), viral, vasculitis or granulomatous disease.
.Typical optic neuritis begins to show improvement with in one month.
Investigation.
If patient recovers most of their vision with in 1-3 months ancillary
testing is usually unnecessary.
Etiology
Idiopathic
Multiple sclerosis
Childhood infections (e.g measles, mumps, chicken pox)
Other viral infections(e.g. herpes zoster)
Contigous inflammation of meninges, orbit,or sinuses
Granulomatous inflammations (e.g. TB, syphilis,
sarcoidosis)
Intraocular inflammation
Indications for Investigation:
 When associated ocular sign such as retinal vasculitis, chorioretinitis
or uveitis present
 Protracted visual loss
 Protracted ocular pain
 Disc swelling
Lab tests:
Serum and CSF VDRL and FTA-ABS for syphilis
Chest X-ray
ESR
Antinuclear antibody testing
MRI
Treatment
 Treat underlying cause.
 Methylprednisolone 250mg q6hrs for 3 days ,followed by oral
Predinsolone 1mg/kg /day for 11days can sped recovery by 1-2 weeks.
 Oral Predinsolone alone has no benefit, and was associated with an
increased rate.
Prognosis:
Visual recovery to 20/40 or better occurs in 92% of patients.
Papilloedema
The term papilloedema refers to edema of the optic nerve head
that results from increased intracranial pressure(ICP).

Symptoms:
 Episodes of transient, often bilateral, visual loss (lasting seconds)
often precipitated by changes in posture
 Those with raised ICP: Headache; double vision; nausea;
vomiting
 Rarely, a decrease in visual acuity ( a mild decrease in VA may
occur in acute setting if associated with a macular disturbance)
 VF defects and severe loss of central VA can occur with chronic
papilloedema
Signs:
Bilaterally swollen, hyperemic discs ( in early
papilledema, disc swelling may be asymmetric) with
blurring of the disc margin, often obscuring the blood
vessels.
Papillary or peripapillary retinal hemorrhages( often
flamme-shaped)
Loss of venous pulsations ( 20% of normal popn. do
not have)
Dilated, tortuous retinal veins
Normal pupillary response and color vision
An enlarged physiologic blind spot
Absence of physiologic cup is a late finding in
papilloedema
Chronic papilloedema
In patient with chronically elevated ICP(months to years) and long
standing papilloedema optic nerve function may deteriorate . The
disc becomes pale from chronic axonal loss(Optic atropy) .
As chronic papilledema progresses to optic atrophy, h’ges &
cotton wool spots resolve, peripapillary gliosis and narrowing
of peripapillary retinal vessels occur.
Loss of color vision, central VA , and peripheral VF also occur.
Dangerous Signs
1) Concentric contraction of visual field

2) Decreased vision

3) Sheathing of Vessels

4) Graying of Disc

5) Increasing Transient Obscurations

Causes of Papilledema
Hypertension
Subarachnoid Hemorrhage
Venous Occlusion
Leukemia
Optic Neuritis with and without decreased acuity
Tumor- optic nerve,orbit,intracranial
Brain Abcess
Juxta Papillary Choroiditis
Posterior scleritis
Work-up
1. Hx & physical exam, including BP measurement
2. Ocular exam including:
 Pupillary exam
 Color vision test
 Posterior vitreous evaluation for cells
 Dilated fundus exam
3. Emergent CT and/or MRI

Treatment
underlying cause of elevated ICP
 Early papilloedema

• VA - normal
• Mild disc hyperaemia
• Indistinct disc margins - initially nasal
• Mild venous engorgement
• Normal optic cup
• Spontaneous venous pulsation - absent (also absent in 20% of norm
Established papilloedema (acute)

• VA - usually normal
• Severe disc elevation and hyperaemia
• Very indistinct disc margins
• Obscuration of small vessels on disc
• Marked venous engorgement
• Reduced or absent optic cup
• Haemorrhages + cotton-wool spots
• Macular star
Longstanding papilloedema (chronic)

• VA - variable
• Marked disc elevation but less hyperaemia
• Disc margins - indistinct
• Variable venous engorgement
• Absent optic cup
Atrophic papilloedema (secondary optic atro

• VA - severely decreased
• Mild disc elevation
• Indistinct disc margins
• Disc pallor with few crossing vessels
• Absent optic cup
Optic nerve atrophy
Optic atrophy refers to the late stage changes that
take place in the optic nerve resulting from axonal
degeneration in the pathway between the retina and
the lateral geniculate body, manifesting with
disturbance in visual function and in the appearance
of the optic nerve head.
Optic atrophy should not be considered a diagnosis; it
is a pathologic end-point that is clinically discernible
but does not imply cause
It can be classified in several ways, including by
whether axonal death is initiated in the retina
(anterograde) or more centrally (retrograde), and
by cause.
Classification of optic atrophy on appearance
Primary optic atrophy
 occurs without antecedent swelling of the optic
nerve head.
 It may be caused by lesions affecting the
visualpathways at any point from the retrolaminar
portion of the optic nerve to the lateral geniculate
body. Lesions anterior to the optic chiasm result in
unilateral optic atrophy, whereas those involving the
chiasm and optic tract will cause bilateral changes.
 Secondary optic atrophy
Secondary optic atrophy is preceded by long-standing
swelling of the optic nerve head.
Consecutive optic atrophy
Consecutive optic atrophy is caused by disease of the
inner retina or its blood supply. The cause is usually
obvious on fundus examination,
e.g. extensive retinal photocoagulation, retinitis pigmentosa
or prior central retinal artery occlusion. The disc appears
waxy, with reasonably preserved architecture
Causes:
Elevated intraocular pressure( glaucoma)
 Systemic hypotension
 Hematologic disorders
 Compression of optic nerve blood vessels
 Degenerative Disease
 Demyelinating diseases
 Idiopathic
 Secondary to
 Trauma
 Compression
 Inflammnation
 Toxic
 Tobacco, alcohol, lead, drugs
 Systemic disease
 Endocrine (Graves' disease, diabetes mellitus)
 Sarcoid
 Blood dyscrasias
 Collagen disease
 Carcinoma
 Starvation (vitamin deficiency)
Cranial
Anatomy
nerve palsy
 The final common pathway for ocular motor control consists of the three
pairs of ocular motor nerves and the muscles that they innervate.
 The nerves originate in paired nuclei within the midbrain and pons, and their
axons course as fascicles through the brain stem parenchyma, run freely for
variable distances within the subarachnoid space, pass through the cavernous
sinus, and enter the orbit to supply the extraocular muscles.
 CN IIImotor innervation for SR, MR, IR, IO, and levator palpebrae
superioris muscles
parasympathetic input to the pupillary constrictor and ciliary muscles
 superior division: SR, levator palpebrae
 Inferior division: MR, IR, IO, Pupil
 CN IVSO
 CN VI  LR
A palsy( weakness) of any one of three motor cranial
nerves (CNs) that supply the extraocular muscles presents
with characteristic findings affecting ocular motility.
A single nerve may be involved; there may be a degree of
bilateral involvement in either the third, fourth, or sixth
CNs; or various combinations of them may be involved in
the lesion.
The palsy may be congenital, due to some defect in the
development of the nucleus or motor nerve fibers, or
acquired.
3rd – nerve palsy
Symptoms:
Double vision that disappears when one eye is closed;
droopy eyelid, with or without pain
Critical Signs:
A. external ophthalmoplegia (i.e., motility status)
Complete palsy: limitation of ocular movement in all fields
of gaze except temporally
Incomplete palsy: partial limitation of ocular movement.
Superior division palsy: Ptosis & inability to look up
Inferior division palsy: Inability to look nasally or inferiorly
; pupil is involved.
3rd – nerve palsy
Critical Signs:
B. Internal ophthalmoplegia (i.e pupil status)
Pupil-involved: A fixed, dilated or minimally reactive pupil
Pupil-spared: pupil not dilated and normally reactive to
light
Relative pupil-spared: pupil partially dilated & sluggishly
reactive to light
Other signs:
Exotropia or hypotropia
Aberrant regeneration
Profound ptosis due to weakness of the levator
muscle.
Abduction and depression in the primary
position (‘down and out’ due to unopposed action
of the lateral rectus and superior oblique muscles.
The intact superior oblique muscle also causes
intorsion of the eye at rest, which increases on
attempted downgaze.
 Normal abduction as the lateral rectus is intact
Limited adduction due to medial rectus weakness
 Limited elevation due to weakness of the superior
rectus and inferior oblique
Signs of third nerve palsy
3 – nerve palsy
rd
Etiology
A.Pupil-involved
 More common: aneurysm (particularly posterior
comminicating artery)
 Less common: microvascular disease( DM, HTN), tumor,

trauma, congenital
 Rare: uncal herniation, cavernous sinus mass lesion,

orbital disease, herpes zoster, leukemia

B. Pupil-spared
 more common: Microvascular disease
 Less common: Cavernous sinus syndrome

C. Relative pupil-spared: Microvascular disease

D. Aberrant regeneration present: Trauma, aneurysm,
tumor, congenital. Not microvascular
3 – nerve palsy
rd

Work-up
Complete ocular exam
Full neurological exam
CT scan/MRI of brain
Cerebral angiography
Immediate ESR if giant cell arteritis is suspected
Rule out myasthenia gravis is suspected
 Anatomy of third nerve
Oculomotor nucleus

Pituitary gland
Red nucleus
Carotid artery

Cavernous sinus

Pons

III nerve
Post cerebral artery Clivus

Basilar artery
Treatment:
Involves relief of the patient's diplopia, which usually is not a
problem in complete third CN paralysis because of the associated
ptosis covering the pupil. However, in partial involvement, the lid
may sufficiently clear the pupillary space so that diplopia is a problem.
Occlusion therapy is the best solution for the patient's diplopia.
Surgery is indicated for associated strabismus and ptosis if the
patient's general condition permits it and if a significant residual
paralysis is present 6 months after onset of third CN palsy
Recovery is usually complete by 6 months following onset,
and, consequently, no judgment should be rendered
regarding the necessity of treatment until after the 6-
month interval.
Partial or complete recovery can be expected, depending
on the etiology of the third CN palsy, in 48% of the
patients.
 Many times such palsy results from relatively serious
intracranial involvement, and this may determine whether
therapy is indicated
Fourth nerve palsy

Internal carotid artery

Postr. communicating
artery
III
VI
Postr.cerebral artery
Supr.cerebellar artery
Basilar artery
IV

• Only cranial nerve to emerge dorsally

• Crossed cranial nerve
• Very long and slender
4th nerve palsy………………..

 The most common isolated cyclovertical muscle palsy encountered is

involvement of the trochlear nerve
Cause
 congenital
 trauma : closed head trauma.
 idiopathic
 Intracranial tumors and aneurysms
 Microvascular (DM. HPN)
 Infectous: cryptococcal meningitis in human immunodeficiency virus
infection causes Bilateral trochlear nerve palsy
4 –nerve palsy
th
 Symptoms
 Binocular vertical diplopia, difficulty reading, sensation that
objects appear tilted; may be asymptomatic
 Signs
The main actions of the superior oblique are intorsion
(incyclo torsion), and depression in adduction
 hypertropia in the primary position
 Limitation of depression, most marked in adduction
 extorsion, greatest in abduction.
 Normal abduction
 Normal elevation
 A compensatory head posture opposite to the side of
palsy
4 –nerve palsy
th

Treatment
Treat the underlying disorder
Patch one eye if symptomatic diplopia
Prisms in spectacles may be prescribed for small
chronic hyperdeviations
 Observation for the first 6 months usually involves simply
waiting to determine the degree of spontaneous recovery.
Surgery
 Bothersome double vision in primary or reading
position, for cosmetic purposes, or for head tilt
 sixth nerve palsy
Basilar artery
Medial Pituitary gland
lemniscus Carotid artery

4th ventricle Cavernous sinus

Petroclinoid
ligament

Vestibular Clivus
VI nerve
nucleus
Pyramidal tract
6 –nerve palsy
th
 Etiology
ADULTS
 More common: vasculopathic( diabetes, HTN,
atherosclerosis), traumatic, idiopathic
 Less common: Increase intracranial pressure, cavernous
sinus mass, multiple sclerosis, sarcoidosis/vasculitis
CHILDREN
 Benign postviral
 Gradenigo’s syndrome (petrositis causing 6th and often
7th nerve involvement, with or without 8th and 5th n.
involvement on the same side)
 Pontine glioma
 Trauma
6th nerve palsy…
 Symptoms
 Binocular horizontal diplopia, worse for distance than near, most
pronounced in the direction of paretic lateral rectus musle
Sign
 Esotropia in the primary position due to relatively
unopposed action of the medial rectus
 Limitation of abduction on the side of the lesion
 Normal adduction of the affected eye
 Bilateral acute sixth nerve paresis is
 considerably less common than unilateral; the causes are broadly similar, but elevated
intracranial pressure should be excluded as a priority.
 A compensatory face turn is towards the side of the paralysed muscle in unilateral

palsy.
 Recent right sixth nerve palsy

Right esotropia in primary position due to

unopposed action of right medial rectus Marked limitation of right abduction du
to
right lateral rectus weakness
 Because of the long intracranial course, angulated over the petrous tip
of the temporal bone, and the absence of any slack in the course
between the brain stem and the dura entry, the
sixth CN is
vulnerable to increased intracranial pressure,
trauma to the cranial floor, meningeal edema,
inflammation in the base of the skull, and any
displacement of the brain stem.
6th –nerve palsy
Treatment
Observation
Occlusion
Surgery in non resolving cases
Nystagmus
Definition
 Nystagmus is the rhythmic to-and-fro oscillation of the eyes
 Symptoms
 Asymptomatic unless acquired after 8 years of age, at which point the
environment may be noted to oscillate horizontally, veritcally, or
torsionally, or vision may seem blurred or unstable
 Signs
 Repetitive oscillations of the eye horizontally, vertically, or torsionally.
 Jerk nystagmus: The eye slowly drifts in one direction(slow phase) &
then abruptly returns to its original position(fast phase), only to drift and
repeat the cycle.
 Pendular nystagmus: Drift occurs in 2 phases of equal speed, giving a
smooth back-and-forth movement of the eye.
 Nystagmus may be congenital or acquired
Nystagmus
A.Congenital
1. Infantile nystagmus
2. Latent nystagmus
3. Nystagmus Blockage syndrome
 Infantile nystagmus
Onset at age 2-3 months , with wide swinging eye movements
jerk and/or pendular pattern
presence with or without normal acuity
conjugate horizontal eye movements that remain
horizontal in up- and downgaze
presence of a null point a position of gaze in which
nystagmus is minimal
no oscillopsia
increasing velocity of slow phase
accentuation by distant fixation; diminishment by
convergence
 Associated head oscillation (not compensatory) or turn
abolished in sleep
Etiology
Idiopathic
Albinism
Aniridia
Leber’s congenitial amaurosis
Others : bilateral optic nerve hypoplasia,
bilateral congenital cataracts, rod
monochromatism, optic nerve or macular
disease
 Treatment
 Maximize vision by refraction
 Treat amblyopia
 If small face turn: prescribe prism in glasses with base in direction of
face turn
 If large face turn: consider muscle surgery
 Latent nystagmus
Clinical characteristics of LN include
conjugate jerk nystagmus
beginning or accentuation when binocular fusion is
disrupted
direction changing with monocular occlusion: fast
phase beats toward viewing eye;
slow phase, toward the nose
congenital esotropia usually present
subnormal stereopsis
Nystagmus with characteristics of LN that is present
when both eyes are open is known as manifest latent
nystagmus (MLN). Because most patients with LN
have esotropia MLN is initiated when the esotropic
eye is physiologically suppressed.
Monocular nystagmus of childhood

is a rare but important form of nystagmus manifests

early in life; causes range from the benign to the
sight- and life-threatening.
 The eye movements are usually in I eye, vertical or
elliptical, and of small amplitude
 often found in concert with an afferent pupillary
defect and optic atrophy, is suggestive of an optic
nerve/chiasmal tumor (glioma), and therefore
neuroimaging is warranted
Management
AIM
 Reduce nystagmus related compensatory head posture by enlarging
the null zone
 And / or dampen the nystagmus intensity
 This may improve the visual acuity
 Patients may find visual quality improved if not the actual acuity
Non – Surgical Management
 Full examination to exclude other ocular or neurological disorders
 Careful refraction
 Optical correction
 Optical image stabilisation systems

Surgery For Nystagmus

 Majority of patients do not benefit from surgery
 Aim of surgery is to relocate the null zone to primary gaze
 Acquired nystagmus

Etiology :
Visual loss(e.g dense cataract, trauma, cone
dystrophy)
Toxic/metabolic
CNS disorders (e.g hemorrhage, tumor,
stroke…)
Treatment
The underlying etiology must be treated.
Pupillary abnormality
Applied Anatomy
The three major optic functions pupil:
 to regulate the amount of light reaching the retina;
 to diminish the chromatic and spherical aberrations
produced by the peripheral imperfections of the optical
system of the cornea and lens; and
 to increase depth of field
Mechanically, the diameter of the pupil is determined by the
antagonistic actions of the iris sphincter and dilator muscles
LIGHT REFLEX PATHWAY

• The pupillary contraction to light is an important neuronal

reflex because it gives information about the integrity of both
the afferent visual system and the efferent neuronal outflow to
each pupil.
3-neuron arc
1. The afferent neurons(sensory) from retinal ganglion cells to
the pretectal area; Impulses originating from the nasal retina
are conducted by fibres which decussate in the chiasm and pass
up the opposite optic tract to terminate in the contralateral
pretectal nucleus. Impulses originating in the temporal retina
are conducted by uncrossed fibres (ipsilateral optic tract)
which terminate in the ipsilateral pretectal nucleus.
  
2. an intercalated neuron from the pretectal complex to
the parasympathetic motor pool (Edinger–Westphal
nucleus) of the oculomotor nuclear complex . Thus a
uniocular light stimulus evokes bilateral and
symmetrical pupillary constriction. Damage to
internuncial neurones is responsible for light-near
dissociation in neurosyphilis and pinealomas
3. The efferent parasympathetic (pre and post
ganglionnic)outflow with the oculomotor nerve to
the ciliary ganglion; and from there to the pupillary
sphincter.
 Near reflex
The near reflex, a synkinesis rather than a true reflex,
is activated when gaze is changed from a distant to a
near target .
It comprises
1)accommodation,
2) convergence and
3) miosis.
Vision is not a prerequisite and there is no clinical
condition in which the light reflex is present but the
near response absent..
Although the final pathways for the near and light
reflexes are identical (i.e. 3rd nerve, ciliary ganglion,
short ciliary nerves), the centre for the near reflex is
ill-defined. There are probably two supranuclear
influences: the frontal and occipital lobes.

 The midbrain centre for the near reflex is probably

located more ventrally than the pretectal nucleus and
this may explain why compressive lesions such as
pinealomas, preferentially involving the dorsal
internuncial neurones involved in the light reflex,
spare the near reflex fibres until later
ABNORMAL PUPILS
Diseases of the visual and nervous system can cause
the pupils to become poorly reactive to light or near
stimuli.
These disorders can be classified into the following
major categories.
1. Afferent Pupillary Defects
2. Efferent Pupillary Defects.
Afferent Pupillary Defects
These are seen in disorders that interfere with the input of
light to the pupillomotor system:
 by blocking light from stimulating the retina;
 by damaging any of the retinal layers; or
 by damaging the optic nerve, chiasm, optic tract, or
midbrain pretectal area.
Afferent pupillary defect
1) Absolute afferent pupillary defect (amaurotic pupil)
 is caused by a complete optic nerve lesion
characterized by the following:   
 The involved eye is completely blind (i.e. no light
perception).
  Both pupils are equal in size.
  When the affected eye is stimulated by light neither
pupil reacts.
 When the normal eye is stimulated both pupils react
normally.
  The near reflex is normal in both eyes
2)Relative afferent pupillary defect (Marcus Gunn pupil
 is caused by an incomplete optic nerve lesion or severe
retinal disease, but never by a dense cataract.
The clinical features are those of an amaurotic pupil but
more subtle.
Thus the pupils respond weakly to stimulation of the
diseased eye and briskly to that of the normal eye.
difference between the pupillary reactions of the two eyes
is highlighted by the ‘swinging flashlight test’ in which a
light source is alternatively switched from one eye to the
other and back, thus stimulating each eye in rapid
succession.
For example in right eye RAPD ……
 When the normal left eye is stimulated, both pupils constrict
 When the light is swung to the diseased right eye, the
stimulus delivered to the constriction mechanism is reduced and both
pupils dilate instead of constricting
 When the normal left eye is again stimulated, both pupils constrict
once more
 •When the diseased right eye is stimulated, both pupils dilate
 It should be remembered that in afferent (sensory) lesions, the pupils
are equal in size; anisocoria (asymmetrical pupil diameter)implies
disease of the efferent (motor) nerve or the iris itself.
This paradoxical dilatation of the pupils in response
to light occurs because the dilatation produced by
withdrawing the light from the normal eye outweighs
the constriction produced by stimulating the
abnormal eye.
It should be emphasized that in afferent (sensory)
lesions, the pupils are equal in size.
 Anisocoria (inequality of pupillary size) implies
disease of the efferent (motor) nerve, iris or muscles
of the pupil.
Afferent Pupillary Defects
Common diseases producing RAPD:
 Dense anterior chamber or vitreous hemorrhage
 Diffuse media opacity( cataract, corneal scar)

 Central or branch retinal artery or vein occlusion

 Retinal detachment

 Anterior ischemic optic neuropathy

 Optic neuritis

 Compressive optic neuropathy

 Chiasmal compression

 Optic tract lesion

 Midbrain tectal damage

Efferent pupillary disorders
Anisocoria: asymetric size of pupil
Etiology of anisocoria:
 The abnormal pupil is constricted
 Unilateral use of miotic drugs e.g pilocarpine
 Iritis
 Horner’s syndrome
 Argyll Robertson pupil
 Long-standing Adies pupil
 The abnormal pupil is dilated
 Iris sphincter muscle damage from trauma
 Adie’s tonic pupil
 Third-nerve palsy
 Unilateral use of dilating eye drop e.g atropine
 Physiologic anisocoria
Physiologic Anisocoria
A normal, asymmetric contribution of the
sympathetic and parasympathetic nervous systems to
each pupil, regardless of ambient lighting.
Anisocoria of around 1 mm is present in around 20%
of the normal population.
The asymmetry persists to the same proportion under
differing levels of illumination.
Oculosympathetic palsy (Horner
syndrome)
The sympathetic supply involves three neurones 1   
First (central) starts in the posterior hypothalamus
and descends, uncrossed, down the brainstem to
terminate in the ciliospinal centre of Budge, in the
intermediolateral horn of the spinal cord, located
between C8 and T2
.
   2    Second (preganglionic) passes from the
ciliospinal centre to the superior cervical ganglion in
the neck. During its long course, it is closely related to
the apical pleura where it may be damaged by
bronchogenic carcinoma (Pancoast tumour) or during
surgery on the neck.
   3    Third (postganglionic) ascends along the
internal carotid artery to enter the cavernous sinus
where it joins the ophthalmic division of the
trigeminal nerve. The sympathetic fibres reach the
ciliary body and the dilator pupillae muscle via the
nasociliary nerve and the long ciliary nerves.
Sympathatic pathway supply
HORNER’S SYNDROME( oculosympathetic defect)
Etiology
First order neuron disorder: stroke, tumor
Second order neuron disorder: tumor(e.g. lung
carcinoma)=>pancoast’s tumor
Third-order neuron disorder: headache syndrome(e.g.
cluster migraine), internal carotid dissection, herpes
zoster virus, otitis media
Congenital Horner’s syndrome: Trauma ( e.g. during
delivery)
 Signs
 The vast majority of cases are unilateral. Causes of bilateral involvement
include cervical spine injuries and as part of systemic autonomic
diabetic neuropathy.   
     Mild ptosis (usually 1–2 mm) as a result of weakness of Müller muscle
 miosis due to the unopposed action of the sphincter pupillae with
resultant anisocoria , accentuated in dim light since the Horner pupil
will not dilate, unlike its fellow.
   Normal pupillary reactions to light and near.
 Hypochromic heterochromia (irides of different colour – Horner is
lighter) may be seen if congenital or long-standing.
   Slight elevation of the inferior eyelid as a result of weakness of the
inferior tarsal muscle
 Reduced ipsilateral sweating, but only if the lesion is below the superior
cervical ganglion, because the sudomotor fibres supplying the skin of
the face run along the external carotid artery.
Rt Horner’s syndrome
Phramacologic tests
Apraclonidine and Cocaine helps to diagnose the
presence of Horner syndrome
 Phenylephrine 1% and adrenaline may be used to
differentiate a preganglionic (abnormal first- or second-
order neurone) from a postganglionic lesion
Apraclonidine 0.5% or 1.0%.
 Result: A Horner pupil will dilate but a normal pupil
is essentially unaffected. The ptosis commonly also
improves.
Explanation: Alpha-1 receptors are upregulated in the
denervated dilator pupillae
Pharmacologic tests
Cocaine 4% is instilled into both eyes.   
  Result: the normal pupil will dilate but the Horner pupil
will not.
   b    Rationale: noradrenaline (NA) released at the post-
ganglionic sympathetic nerve endings is re-uptaken by the
nerve endings, thus terminating its action. Cocaine blocks
this uptake. NA therefore accumulates and causes pupillary
dilatation. In Horner syndrome, there is no NA being
secreted in the first place – therefore cocaine has no effect.
Cocaine thus confirms the diagnosis of Horner syndrome by
continued constriction of the affected pupil.

  
 Phenylephrine 1%
Result: In postganglionic lesion, the Horner pupil will
dilate and ptosis may be temporarily relieved.
A central or preganglionic Horner pupil and a normal
pupil will not dilate
Explanation: In postganglionic Horner syndrome the
dilator pupillae muscle develops denervation
hypersensitivity to adrenergic neurotransmitters due
to its dysfunctional local motor nerve
Adrenaline has similar response to that of
phenylephrine
ADIE’S TONIC PUPIL
is caused by denervation of the postganglionic
parasympathetic supply to the sphincter pupillae and the
ciliary muscle, and may follow a viral illness.
 It is occasionally inherited in an AD pattern. Sites of
dysfunction are presumed to be the ciliary ganglion,

Damage to the ciliary ganglion or the short ciliary nerves

may cause a tonic pupil It
typically affects young women and presents in one eye in
80%, though involvement of the second eye typically
develops within months or years.
Symptoms:
Difference in the size of the pupils, blurred vision for
near sue to affected accomodation may be
asymptomatic
ADIE’S TONIC PUPIL
Signs:
 An irregularly dilated pupil exhibiting minimal or no
reaction to light, slow constriction to convergence , and slow
redilatation.
On slit lamp examination, vermiform movements of
the pupillary border are typically seen.
 The pupil demonstrates supersensitivity to weak cholinergic
agents(e.g. pilocarpine 0.125%)
 The pupil dilates normally to mydriatic agents.
 The involved pupil may become smaller than the normal
pupil over time
 Deep tendon reflexes (knee and ankle) are often
absent( Adie’s syndrome)
ADIE’S TONIC PUPIL
Etiology
Idiopathic, orbital trauma or infection, herpes zoster
infection, diabetes, autonomic neuropathies, others
Work-up
Observe the suspected pupil with slit lamp. The Adie’s
pupil will contract slowly and irregularly.
Test for supersensitive pupil: instill a drop of
pilocarpine 0.125% in each eye. Check after 15 min. The
tonic pupil constricts significantly more than the
contralateral pupil in Adie’s syndrome. (‘little old Adie’)
Argyll Robertson pupils
are caused by neurosyphilis, and have been attributed to a dorsal
midbrain lesion that interrupts the pupillary light reflex pathway
but spares the more ventral pupillary near reflex pathway– light–
near dissociation results.
are caused by neurosyphilis and are characterized by   
a)    In dim light both pupils are small and may be irregular.
   b)    In bright light neither pupil constricts.
   c )   On accommodation both pupils constrict (light-near
dissociation).
   d  )  After instillation of pilocarpine 0.1% into both eyes,
neither pupil constricts helps to distinguish it from long
standing adies pupil