4

22 Neuro-ophthalmology
and Squint

High Yield Facts

• Most common cause for optic neuritis is Multiple sclerosis.

• Type of optic atrophy seen in retinitis pigmentosa is consecutive optic atrophy.
• Type of field loss seen in tobacco amblyopia – centrocaecal scotoma.
• Most common cause for vertical diplopia is superior oblique palsy.
• Binocular vision is tested by synaptophore.
• Effective treatment for amblyopia is occlusion therapy.

Visual Pathway zz It consists of the axons originating from the ganglion

cells. It consists of 1.2 million axons#
Visual pathway consists of: zz It is a white matter tract of central nervous system
zz Optic nerve covered by meninges#.
zz Optic chiasma zz 40 – 70 mm in length and divided into 4 parts
zz Optic tract  Intra Ocular – 1mm
zz Lateral geniculate body  Intra Orbital – 30 Mm
zz Optic radiation  Intra Canalicular – 6- 10 Mm
zz Visual cortex  Intra cranial – 10 mm
zz Myelination starts from the brain and ends just behind
Optic Disc/Papilla/Optic Nerve Head the lamina cribrosa#.
zz Optic disc measures about horizontally 1.5mm.
zz Optic nerve head corresponds to physiological blind Optic Chiasma
spot.
zz Chiasma means cross road.
zz Blood supply to the optic nerve head#:
zz 53% nasal fibres crosses and temporal fibres remain
 Prelaminar region: Centripetal branches from the
ipsilateral.
peripapillary choroid
zz 12mm (horizontally) and 8mm (anteroposteriorly)
 Laminar region: from the Posterior ciliary artery
zz 10 mm above the sella turcica
and The circle of Zin.
zz Anteriro jenu/Wilbrandt’s knee#: While crossing over, the
 Retrolaminar region: Centrifugal branches from
inferonasal fibres pass through the contralateral optic
Central Retinal artery and the centripetal branches
nerve and then enter onto the optic tract.
from the pial plexus

Optic Nerve Optic Tract

zz 2nd cranial nerve; has only sensory component. zz Each optic tract consists of fibres from the temporal half
zz Starts from the optic disc, extends to the optic chiasma of the retina of the same eye and the nasal half of the
where two nerves meet and relay at the LGB opposite eye.
 Triple O

zz Posteriorly each optic tract ends in the lateral geniculate zz Optic radiations consist of the axons of third order
474 body. neurons in the LGB.
zz The pupillary reflex fibres pass through the superior zz Pass forwards and laterally as OPTIC PEDUNCLE,
brachium → Pretectal nucleus in the mid brain. anterior to the lateral ventricle and traversing close to the
zz Although the optic nerve anatomically ends at the optic internal capsule.
chiasma, the retinal ganglion axons continue within the zz The Superior fibres pass through the parietal lobe and
optic tract until lateral geniculate nucleus. finally end in the visual cortex.
zz The Inferior fibres pass through the temporal lobe and
then into the visual cortex.
Lateral Geniculate Body
Each geniculate body consists of six layers of neurons
zz
(grey matter) alternating with white matter (formed by
Visual Cortex
optic fibres). zz Situated on the medial aspect of the occipital lobe - in
zz The fibres of second-order neurons coming via optic and around the calcarine fissure
tracts relay in these neurons. zz Sub divided into
zz Uncrossed fibres relay in – 2, 3, 5 layers; Crossed fibres  Visuosensory Area – 17#
relay in– 1, 4, 6 layers.  Visuopsychic area – 18 and 19#
zz Macular fibres: Posteriorly represented in the occipital
cortex.
Optic Radiations zz Peripheral retina: Anterior to the macular fibres.
zz The fibres from the LGB ends in the visual area of the zz Uniocular fibres: Anterior to this.
cerebral cortex.
TABLE 1: Site of lesion in the visual pathway and field defects

Site Field Defects Other Features

Optic nerve Total loss in one eye

Proximal part of optic JUNCTIONAL SCOTOMA – anopia in ipsilateral

nerve eye and temporal hemianopia
In the CL eye
Optic chiasma Bitemporal / binasal hemianopia
Optic tract CL incongruous homonymous hemianopia • Wernicke’s hemianopic pupil

LGB CL incongruous# homonymous hemianopia

Optic radiation in CL congruous homonymous inferior • Opto kinetic response (OKN) asymmetric
parietal lobe Quadrantonopia • Acalculia, agraphia, finger agnosia (Gerstmann
Pie in the floor syndrome), left –right disorientation, spatial neglect#
Optic radiation in CL congruous homonymous superior • FORMED visual hallucinations
temporal lobe Quadrantonopia • Olfactory (UNCINATE FITS) and gustatory hallucination
Pie in the sky • Dejavu phenomenon
Visual cortex CL congruous homonymous hemianopia • Unformed visual hallucination
Macular splitting / macular sparing • ANTON syndrome – denial of blindness
OPHTHALMOLOGY

• RIDDOCH phenomenon – static target not visualised but

kinetic target is visualised.

zz Cortical blindness – Bilateral occipital cortex involvement

zz Characterized by bilateral loss of vision, normal pupillary light
reflex and normal fundus

Fig. 1: Visual pathway and field defects
zz WERNICKE’S HEMIANOPIC PUPIL: Due to damage
of the optic tract, there is loss of pupillary constriction
when the light is directed to the blind side of the
retina; pupillary constriction is maintained when light
stimulates the normal side.
zz SPIRALLING VISUAL FIELD# / concentrically contracting
field is seen in HYSTERICAL BLINDNESS.
Normal Pupillary Reflexes
zz Light reflex: When light is shown into the light it causes
constriction of pupil.
Two Types of Light Reflex
zz DIRECT LIGHT REFLEX: When light is shown in the one
eye, constriction of the pupil in the same eye is called
Direct light reflex.
zz Indirect or consensual pupillary light reflex in which the
opposite pupil will constrict. The reason for consensual
light reflex is decussation of nasal fibers at optic chiasma
and decussation of fibers at Edinger - Westphal nucleus.
Normal light reflex pathway as follows
zz Retina: Optic nerve fibers → Optic Chiasm → Optic
tract → Pretectal nucleus → Edinger Westphal nucleus
→ fibres joins the parasympathetic fibers join the III
nerve → from Ciliary ganglion → Short ciliary nerves →
Sphincter pupillae.
Chapter 22  |  Neuro-ophthalmology and Squint
475
Fig. 2: Pupillary pathway
zz Near reflex: It comprises Accommodation, Convergence
and Miosis#.
zz Afferent fibres from the medial rectus via third nerve →
the Mesencephalic nucleus# of fifth nerve to convergence
center in the tectal or pre-tectal region → Edinger –
Westphal nucles → then similar to pupillary light reflex
pathway.
There are two types of muscles in the iris
zz SPHINCTER PUPILLAE → Constrict the pupil → supplied by
parasympathetic fibers via III cranial nerve
zz DILATOR PUPILLAE → dilate the pupil → supplied by
sympathetic plexus from the cervical ganglion.
Pupillary Abnormalities
MARCUS GUNN PUPIL: Relative Afferent pupillary defect
OPHTHALMOLOGY
zz Seen in incomplete Optic nerve / retinal disease.
zz SWINGING FLASH LIGHT TEST#: When the light is
swung between the two eyes, instead of constricting the
pupil dilates on the affected side.
zz Earliest indicator of optic nerve disease#.
AMAUROTIC LIGHT REFLEX: Afferent Pupillary defect
zz Absence of direct light reflex on the affected side.
zz Seen in Complete optic nerve / retinal disease.
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Efferent Pathway Defects

476 zz Horner’s syndrome
zz Holmes Adie pupil
zz Argyll Robertson pupil
TABLE 2: Features of Pupillary abnormalities

Features Horner’s Syndrome Adie’s TONIC pupil Argyl Robertson Pupil

Site of lesion Sympathetic pathway in head Ciliary ganglion or short Dorsal midbrain (pretectal interneurons to Edinger
and neck ciliary nerves Westpaul nucleus involved, sparing ventrally placed
accommodative reflex neurons)
Size Small Dilated Small, irregular
Laterality Mostly unilateral Mostly unilateral Bilateral and symmetrical
Bilateral Horner’s is seen
in cervical spine injury and
diabetic neuropathy
Light reflex + • Slow reaction to light# Absent
• Vermiform movements#
of the pupillary margin
Accommodation + Reacts slowly to near, Present
reflex redilation is also slow
[TONIC pupil]
Associated Holmes Adie syndrome – Dorsal midbrain syndrome - Syphilis
conditions diminished deep tendon
reflexes
Pharmacological Cocaine- no dilatation 0.125% pilocarpine – Cocaine – dilatation
tests constriction 0.125% pilocarpine – no constriction

Hutchinson’s Pupil (demyelination of the spinal cord) within days or

Irritative miosis followed by mydriasis due to compression of weeks.
the Third CN in head injury. zz Schilder disease, a progressive generalized disease with
an onset prior to the age of 10 years and death within
1–2 years. Bilateral optic neuritis without subsequent
Optic Neuritis improvement may occur.
zz Parainfectious infection – Follow a viral infection and
Etiology immunization
zz Inflammation of the optic nerve which includes zz Infectious: Sinus related, Systemic infections – Cat
demyelinating disease or the inflammation from the scratch fever, Syphilis, Lyme disease, Herpes zoster
orbital or paranasal sinuses. zz Non-infectious: Sarcoidosis
zz It can affect the optic nerve from the optic disc to the zz Autoimmune diseases such as SLE, PAN and other
LGB. vasculitides
OPHTHALMOLOGY

Etiology Symptoms
zz Demyelinating disease – Most common cause - Multiple zz Sudden painful loss of vision → Deep orbital or brow
Sclerosis* pain#
zz Painful EOM due to the involvement of the origin
Other Rare Causes of superior rectus and medial rectus [Whitnall’s
hypothesis]
zz Devic disease (neuromyelitis optica)#, occur at any zz Defective colour vision especially red color#
age, characterized by bilateral optic neuritis and the
subsequent development of transverse myelitis#
 Chapter 22  |  Neuro-ophthalmology and Squint

Signs
zz VA – moderate loss 6/60
477
zz Local tenderness
zz Defective colour vision disproportionate to the loss of
vision#
zz Loss of contrast sensitivity
zz Movement phosphenes and sound induced phosphenes
(glowing sensations)#
zz Worsening of symptoms following exercise or increased
body temperature: UHTHOFF’S SIGN#
zz Altered perception of moving objects that results in the
stereoillusion -PULFRICH’S PHENOMENON#
zz PAROXYSMAL CONVERGENCE SPASM: Rare but
typical symptom in MS due to abnormal EXCITATION.
In MS, not only delay in conduction occurs, sometimes
spontaneous excitation occurs. Due to demyelination
plaque in Medial longitudinal fascilculus.
zz Field defects – Central or centrocaecal scotoma#
zz RAPD [Marcus Gunn pupil]

TABLE 3: Types of optic neuritis (Based on anatomical location):

Fig. 3: Optic neuritis – MRI – Dawson’s finger
Types Part of Fundus Picture
Optic Nerve
Papilloedema
Involved PAPILLOEDEMA is a passive bilateral, non inflammatory,
Papillitis Optic disc • Disc edema and hyperemia non ischemic disc swelling associated with increased Intra
(alias papilla) with obliteration of the – Cranial Tension.
optic cup zz It is always bilateral,
• Peripapillary flame shaped zz It may be asymmetrical#
hemorrhage
• Inflammatory cells in the Causes
posterior vitreous
zz Primary and metastatic intracranial tumors (Posterior
Neuro- Optic disc and • PAPILLITIS + MACULAR
cranial fossa tumours).
retinitis retina STAR
zz Aqueductal stenosis producing hydrocephalus.
• Visual loss is mainly due to
the macular edema#
zz Pseudotumor cerebri (Often occurs in young, overweight
females)#.
Retro-bulbar Optic nerve • Optic disc normal and zz Subdural and epidural hematomas (From trauma.).
optic neuritis (behind the RNFL normal zz Subarachnoid hemorrhage
eyeball) • “Pt Sees Nothing and Also zz Arteriovenous malformation.
Doctor Sees Nothing” zz Brain abscess
zz Meningitis
Management zz Encephalitis
Intracranial venous sinus thrombosis.
OPHTHALMOLOGY
zz
zz Treat the primary cause.

Optic Neuritis Treatment Trial: (ONTT Trial)

zz IV methyl prednisolone 250 mg 6th hourly for 3 days →
followed by oral prednisolone 1mg/kg/day for 11 days.
zz Prednisolone is rapidly tapered over the next 3 days.
zz Hastens the visual recovery and decreases the
recurrence but the long term visual outcome is no
different from that achieved by observation alone#.
Foster Kennedy syndrome
zz Associated with olfactory or sphenoidal meningiomata and
frontal lobe tumors
zz Pressure optic atrophy on the side of the lesion
zz Papilledema on the other side due to increased ICT
Pseudo Foater Kennedy syndrome
zz Like in recurrent Multiple Sclerosis
 Triple O

Hayrey’s Theory
478 Increased ICT → Transmitted elevated CSF pressure in the
sub arachnoid space around the optic nerve → Interrurpted
orthograde axoplasmic outflow → Nerve fibre layer edema
→ Venous congestion → PAPILLOEDEMA

Clinical Features
zz Initial stages, optic nerve functions will be normal.
Once the atrophy sets in, VA decreases and field
defects occur.

Fig. 4: Established papilloedema with Paton’s line

TABLE 4: Stages of papilloedema and its clinical features

Stage Vision Pupil Optic Disc Changes

EARLY PAPILLLOEDEMA Normal Normal Nasal margin blurring (EARLIEST SIGN), Hyperemia, loss of
spontaneous venous pulsations
ESTABLISHED PAPILLOEDEMA Transient visual Normal Disc edema (> 3 DD), Venous tortuosity, hemorrhages, CWS,
obscurations physiological cup obliterated, Paton’s line
Enlarged blind spot
CHRONIC / VINTAGE VA impaired APD Pallor starts,
PAPILLOEDEMA Peripheral VF “CHAMPAGNE CORK” appearance of the disc
constricted CORPORA AMYLACEA# – drusen like deposits over the disc
ATROPHIC PAPILLOEDEMA VA and VF severely APD Secondary optic atrophy
impaired
Paton’s line#: Disc edema extending into the surrounding retina forming folds concentric to it called Paton’s line.

Causes of Disc Edema

TABLE 5: Disc edema - Causes

Unilateral
zz Papillitis or optic neuritis involving the nerve head (sudden loss of vision with subsequent improvement)
zz Anterior Ischaemic Optic neuropathy (sudden loss of vision usually without improvement)
zz Central retinal vein occlusion
zz Ocular hypotony
zz Foster- Kennedy Syndrome (true papilloedema in one eye with optic atrophy in the fellow eye)
zz Pseudo foster Kennedy Syndrome
OPHTHALMOLOGY

Bilateral
zz Papilloedema
zz Hypertension
zz Diabetic papillopathy
zz Cavernous sinus thrombosis
zz Carotid-cavernous fistula
zz Leber Hereditary optic neuropathy in the acute stage
 Chapter 22  |  Neuro-ophthalmology and Squint

Optic Atrophy
It is the pallor of the optic disc due to death of nerve fibers due to damage at any point from the ganglion cells to the lateral
479
geniculate body.
TABLE 6:  Types of optic atrophy and its clinical features#

Primary optic atrophy
(poa)
Site of lesion Damage occurs behind the
eye
DISC • CHALKY WHITE DISC
APPEARANCE ƒƒ Well defined margins
ƒƒ With surrounding
normal retina
CAUSES Pituitary tumor, tumor of
the optic nerve, optic nerve
injury, retrobulbar optic
neuritis
Secondary optic atrophy
Followed by disc edema
• DIRTY WHITE in colour
ƒƒ Margins blurred
ƒƒ Perivascular sheathing
ƒƒ With surrounding
normal retina
Papilloedema, papillitis,
neuroretinitis
Consecutive optic Glaucomatous optic
atrophy atrophy
Secondary to retinal Due to increased IOP
diseases
• YELLOW WAXY DISC • Normal colour with
ƒƒ Margins blurred well defined margins
ƒƒ Retinal pathology • DEEP CUP
Retinitis pigmentosa, Glaucoma,
CRAO, choroiditis, post Methanol poisoning
PRP

Leber’s Hereditary Optic Neuropathy (LHON)

zz Mitochondrial inheritance#
zz Only males are affected#
zz Optic Disc - Hyperemia with telangiectasia →resulting in Optic atrophy
zz FFA : NO leakage from the disc or the telangiectatic vessels
zz Pupillary reaction may remain fairly brisk
WOLFRAM SYNDROME (DIDMOAD): Autosomal recessive
zz DI: Diabetes Insipidus
zz DM: Diabetes Mellitus
zz OA: Optic atrophy
zz D: Deafness
TABLE 7: Typical Visual Field Defects

Tubular vision Enlargement of blind spot Central scotoma

• Terminal stage of glaucoma • POAG • Optic neuritis
• Advanced stage of RP • Papilledema • Macular hole, cyst degeneration
• Chronic simple glaucoma • Medullated nerve fibers
• Quinine poisoning • Drusen of ON
• High myopia • Juxtapapillary choroiditis
• CRAO with sparing of cilioretinal Ring scotoma
artery
• POAG
OPHTHALMOLOGY
• RP
• High myopia
• PRP
• Aphakia with spectacle correction
 Triple O

TOXIC/NUTRITIONAL OPTIC NEUROPATHY

480 Tobacco mainly affects the PAPILLOMACULAR BUNDLE leading to temporal pallor of the disc .
TABLE 8: Toxic amblyopia – clinical features

Toxic agents Pathogenesis Clinical features Management

TOBACCO – ALCOHOL • Ganglion cells of the macular Gradual painless defective vision Abstinence from tobacco
AMBLYOPIA region is affected. Temporal pallor (papillomacular Vitamin B12 1000 microgram i.m.
• Cyanide is the main toxic bundle) of the disc weekly for 10 weeks
agent Centroceacal scotoma
METHANOL Methanol → Formaldehyde → Systemic : Headache, nausea, Gastric lavage
Intake of wood/adulterated Formic acid vomiting, abdominal pain, Soda bicarbonate# (500ml of 5%)
alcohol, inhalation of the These products get delirium, stupor and even death oral / I.V to overcome acidosis
fumes accumulated# in the ganglion Ocular: Sudden onset of Ethyl alcohol – competes with the
cells → degeneration and optic Complete blindness alcohol dehydrogenase enzyme
atrophy • Mild disc edema with since it has more affinity
attenuated vessels → bilateral Peritoneal dialysis#
Primary Optic atrophy Poor prognosis

Ethambutol
zz Dose dependent toxicity. Risk increases when associated
diabetes and alcoholism is present. AMAUROSIS FUGAX
 Presents as OPTIC NEURITIS#
Transient visual obscurations
 Recovers after stopping the drug.
Causes:
Carotid TIA, emboli in retinal circulation, papilloedema, Giant
cell arteritis, Raynaud’s disease, migraine, HTR, Venous stasis
Anterior Ischaemic Optic retinopathy
Neuropathy (AION)
zz Optic nerve head ischemia due to Short posterior
ciliary artery occlusion#
Giant Cell Arteritis/Temporal Arteritis:
Two Types zz Age: 70 years, females are commonly affected
zz Arteritic – Giant Cell Arteritis (GCA) zz Jaw claudication (cramp-like pain on chewing)#, caused by
ischaemia of the masseter muscles, is virtually pathognomonic.
zz Non – arteritic → small disc (disc at risk), HTN, DM
zz OCULAR FEATURES: Amaurosis fugax, Arteritic anterior isch-
aemic optic neuropathy, Diplopia.
Clinical Features zz Erythrocyte sedimentation rate of 50 mm/hr or greater.
zz Old age zz FELLOW EYE INVOLVEMENT CAN OCCUR IN 30% OF CASES
WITHIN ONE WEEK → IV methyl prednisolone should be
zz Sudden onset of mono ocular field loss (INFERIOR/
started as early as possible.
SUPERIOR ALTITUDINAL HEMIANOPIA#)
zz VA – 6/60
zz Colour vision defect is proportional to the vision loss OCULOMOTOR NERVE PALSY
OPHTHALMOLOGY

zz RAPD
FUNDUS: PALID HYPEREMIC DISC EDEMA with splinter Abducent Nerve
hemorrhage in the disc
zz Sixth cranial nerve palsy Causes Lateral rectus paralysis
→ Horizontal diplopia
Treatment
zz Non-arteritic type- Treat the systemic cause Causes
zz Arteritic type - IV Methylprednisolone + oral steroids
zz Ischemic mononeuropathy due to DM, HTN.
zz As a false localizing sign due to increased intracranial
pressure.
 Chapter 22  |  Neuro-ophthalmology and Squint

Internuclear Ophthalmoplegia
zz Horizontal gaze center is pontine paramedian reticular
481
False Localising Signs formation.
Occurs to distortion or displacement of the brain tissue due to zz Impulses from PPRF → Ipsilateral 6th nerve nucleus →
increased ICT. ipsilateral abduction
zz Diplopia – 6th nerve paralysis zz Through Medial longitudinal fasciculus MLF → crosses
zz Sluggish pupillary reflex and unilateral mydriasis. the midline → Contralateral 3rd nerve Medial rectus
zz Bitemporal hemianopia – chiasma compression by the subnucleus → contralateral adduction.
distended 3rd ventricle
zz Homonymous hemianopia – occipital lobe herniation Internuclear Ophthalmoplegia
zz Lesion in the MLF#; Cause - demyelination, stroke and
Trochlear Nerve tumours.

It is the only cranial nerve to emerge from the dorsal

Clinical Features
zz
aspect of the brain.
zz It is the only decussated (crossed) cranial nerve zz Ipsilateral adduction is defective with clntralateral
besides the optic nerve, innervating the superior oblique abducting nystagmus.
muscle contralateral to its nucleus. zz Gaze to the side of the lesion is normal.
zz Convergence is intact
Longest Intracranial Course
zz The fourth (trochlear) cranial nerve supplies only the
One and Half Syndrome
superior oblique muscle, so the fourth nerve palsy zz Ipsilateral PPRF and MLF lesion#
presents with vertical diplopia/ difficulty while climbing zz Ipsilateral abduction and adduction is lost( ONE ) ;
down the stairs. contralateral adduction is affected (HALF )  ONE AND
zz BEILSCHOWSKY TEST is done to confirm Fourth CN HALF SYNDROME
palsy. zz Only contralateral abducting nystagmus is present.

Oculomotor Nerve
Extra Ocular Movements and
The most common cause of Isolated third nerve palsy
zz
- Posterior communicating aneurysm at the junction
Binocular Single Vision
of ICA Orthotropia - Definition
zz Pupil sparing Third cranial nerve palsy – suspect
ischemic mononeuropathy zz Perfect alignment of the eye in the absence of any visual
zz Pupil involving Third cranial nerve palsy – suspect stimulus for fusion.
surgical causes like PCA aneurysm, head injury.

Syndromes Associated with Third Cranial Nerve

zz WEBINO#: Involvement of the paired medial rectus
subnuclei, characterized by exotropia with defective
convergence and adduction.
OPHTHALMOLOGY
zz Benedikt syndrome#: red nucleus → ipsilateral third
nerve palsy and contralateral extrapyramidal signs such
as hemitremor.
zz Weber syndrome#: the cerebral peduncle ipsilateral
third nerve palsy and a contralateral hemiparesis.
zz Nothnagel syndrome#: the superior cerebellar peduncle
→ ipsilateral third nerve palsy and cerebellar ataxia.
zz Claude syndrome# is a combination of Benedikt and
Nothnagel syndromes.

Fig. 5: Right INO

 Triple O

Extraocular Muscles – Anatomy and Physiology

482 TABLE 10: Origin and insertion of the EOM

Origin Insertion Length Tendon

length
Medial Annulus of 5.5 mm 40 mm 3.7 mm
rectus zinn from the
limbus
Inferior Annulus of 6.5 mm 40mm 5.5 mm
rectus zinn
Lateral Annulus of 6.9 mm 48mm 8.8 mm
rectus zinn
Superior Annulus of 7.7 mm 42 mm 5.8 mm
rectus zinn
Fig. 6: Left one and half syndrome Superior Lesser wing 16.3 mm 60 mm 20 mm
oblique of sphenoid
zz Visual axis of the two eye are parallel to each other in Inferior Orbital 18.4 mm 37 mm 1-2 mm
the primary position of gaze and this alignment is main- oblique part of the
tained in all positions of gaze. maxilla

HIRSHBERG Test
zz Ocular alignment is assessed by Hirshberg test. zz Longest and thinnest muscle is superior oblique#
zz Torch light is shown from 33cm over the glabella and ask zz Shortest muscle is inferiro oblique#
the patient to look at the torch. Observe the corneal light
reflex. zz The insertions are located progressively further away
from the limbus in a spiral pattern; the medial rectus
TABLE 9: Hirshberg test and its interpretations insertion is closest (5.5 mm) followed by the inferior
Position of the corneal light Ocular alignment rectus (6.5 mm), lateral rectus (6.9 mm) and superior
reflex rectus (7.7 mm)
In the center of the pupil Orthotropic
Spiral of Trillaux#
Medial to the pupil Exotropia / Divergent squint
Lateral to the pupil Esotropia / Convergent squint zz An imaginary line joining the insertions of the four recti.
zz An important anatomical landmark when performing
For 1mm displacement of corneal light reflex corresponds to surgery
7* of squint.
OPHTHALMOLOGY

Fig. 7: Hirshberg Test Fig. 8: Spiral of Trillaux

 Chapter 22  |  Neuro-ophthalmology and Squint

Axes of Fick Gaze Centers

zz VERTICAL → Z axis TABLE 12: Gaze centers and their anatomical location
483
zz HORIZONTAL → X axis
Action Nucleus
zz ANTERO POSTERIOR → Y axis
zz The plane which passes through the center of rotation of Horizontal gaze center Pontine paramedian reticular
the eye is called LISTING’S PLANE. formation (PPRF)
Vertical gaze center Interstitial nucleus of Cajal
Actions of the Muscle Convergence center Nucleus of Perlia
Pneumonic is SIN RAD – all Superiors are INtorters, all Recti Divergence center Exists possibly
are ADductors Parasympathetic to the eye Edinger Westpaul nucleus
OBS ABS – all OBliques are ABductors
Sympathetic to the eye Hypothalamus
TABLE 11: Extraocualr muscles and its actions Contralateral saccades Frontal and parietal lobe
Muscle Primary Secondary Tertiary Ipsilateral smooth pursuit Temporo occipital junction /
action action action occipital lobe
Medial rectus Adduction ----- -----
Lateral rectus Abduction ----- ----- Ocular Motility
Superior Elevation Intorsion Adduction Types of Extraocular Movements
rectus
Ductions
Inferior Depression Extorsion Adduction
rectus zz Monocular movements around the axes of Fick#.
Superior Intorsion Depression Abduction zz ACTIONS : Adduction, Abduction, Elevation, Depression,
Oblique Intorsion, Extorsion.
zz They are tested by occluding the fellow eye and asking
Inferior Extorsion Elevation Abduction
the patient to follow a target in each direction of muscle
Oblique
action.

Nerve Supply Versions

zz Pneumonic is LR6 SO4 remaining 3 zz Versions are binocular, simultaneous, conjugate#
zz Lateral rectus by Abducent nerve movements in the same direction#
zz Superior Oblique by Trochlear nerve zz Actions: Dextroversion, Dextroelevation, Dextro-
zz MR, SR, IR, IO, Levator Palpebrae superioris by Oculo- depression (RIGHT SIDE gaze)
motor nerve.  Levoversion, Levoelevation, Levodepression (LEFT
SIDE gaze)
 Supraversion, Infraversion
 Dextrocycloversion, Levocycloversion

Vergences
zz Binocular, simultaneous, disjugate or disjunctive
movements (in opposite directions)
OPHTHALMOLOGY
zz CONVERGENCE is simultaneous adduction (inward
turning)
zz DIVERGENCE is outwards movement from a convergent
position.

Laws of Ocular Motility

Agonist–Antagonist Muscles
zz Pairs of muscles of the same eye that move the eye in
Fig. 9: Axes of Fick
opposite directions.
 Triple O

 Agonist is the primary muscle moving the eye in a Binocular Single Vision
484 given direction.
zz Co-ordinated use of both the eyes so as to produce a
 Antagonist acts in the opposite direction to the
agonist. single mental impression.
 E.g.: Right lateral rectus is the antagonist to the right
medial rectus. Grades
zz Grade I – SIMULTANEOUS MACULAR PERCEPTION
Synergists zz Grade II – FUSION
zz Muscles of the same eye that move the eye in the same zz Grade III – STEREOPSIS
direction.
zz Right superior rectus and right inferior oblique act Importance of Stereopsis and Binocular Single Vision
synergistically in elevation. zz Increase field of vision
 Eliminate the blind spot – the blind spot of an eye
Yoke Muscles (Contralateral Synergists) fall on the opposite eye’s visual field.
 Binocular acuity is greater than monocular
zz Pairs of muscles, one in each eye, that produce conjugate  Depth perception
ocular movements.  Estimation of Distance
zz The yoke muscle of the left superior oblique is the right Test for Binocular Vision#
inferior rectus.  Worth’s four dot test
 Neutral density filter test
Hering Law of Equal Innervation  Bagolini striated glasses
During any conjugate eye movement, equal and simultaneous
innervation flows to the yoke muscles.
Strabismus (Squint)
Sherrington Law of Reciprocal Innervation zz Misalignment of visual axis of both the eyes
(Inhibition)
zz Increased innervation to an extraocular muscle (e.g. right
medial rectus) is accompanied by a reciprocal decrease zz Convergent squint (Esotropia) causes Uncrossed diplopia
in innervation to its antagonist (e.g. right lateral rectus) (Homonymous)
zz When the medial rectus contracts the lateral rectus zz Divergent squint (Exotropia) causes Crossed diplopia
automatically relaxes and vice versa.
zz Applies to both versions and vergences.
Squint
zz Misalignment of visual axis of the two eyes.

Classification
zz Apparent squint / pseudostrabismus
zz Latent / heterophoria
zz Manifest / heterotropia
 Concomitant
Incomitant
OPHTHALMOLOGY



TABLE 13: Investigations for Squint

To detect squint To measure the amount of

deviation
• Hirshberg test • Prism Bar Test → Apex Towards The
• Cover test Deviation
• Cover – uncover test • Maddox Wing Test
• Synoptophore – BSV also tested.

Fig. 10: Yoke muscles

 Chapter 22  |  Neuro-ophthalmology and Squint

485

(A)

Fig. 12: Krimski test

zz Pseudoesotropia: Epicanthal fold, Negative angle

kappa, Myopia
zz Pseudoexotropia: Hypertelorism, Positive angle kappa,
Hypermetropia

(B) Latent/Heterophoria
zz No deviation on seeing the eyes. When the fusion is
removed, squint is manifested.
zz FUSIONAL REFLEXES play a major role in keeping the
eye in orthotropic position. Possible only in small angle
squints#.....
Fig. 11: (A) Cover test (B) Prism Bar test zz Hirshberg test → normal
zz Cover test → normal
Microtropia zz Cover uncover test → abnormal
zz Commonest latent squint – convergence insufficiency
zz Small Esotropia of < 10 prism dioptres or 5°
KRIMSKI’S TEST# – done to detect microtropia using
single prism Manifest Squint
zz Types: Concomitant and Incomitant squint.
Apparent Squint/Pseudostrabismus
zz On seeing the patient, eye seems to have squint but on Causes
examining, ocular alignment is normal.
zz Hirshberg test → normal Concomitant Squint
zz Cover test → normal
zz Decreased vision like refractive error, cataract
zz Central fusional reflex not formed / broken – CP,MR
OPHTHALMOLOGY

zz Ocular muscle imbalance

Angle Kappa
Incomitant Squint
Angle between visual axis and pupillary plane.
zz +ve angle kappa is seen in Pseudoexotropia zz Restrictive: Thyroid eye disease, myositis, orbital space
zz -ve angle kappa is seen in Pseudoesotropia occupying lesions, fibrosed muscle
zz Paralytic: 3, 4, 6 cranial nerve paralysis
 Triple O

TABLE 14: Difference between concomitant and incomitant Surgery

486 squint
zz Muscle Recession (weakening), Muscle Resection (Stre-
Concomitant Incomitant ngthening) procedures are done.
Squint Squint
Brown Syndrome
MAGNITUDE OF Same in all Varies with eye
SQUINT positions position zz Restrictive type of squint
zz Upward gaze impairment while the eye is in adduction
OCULAR No limitation Restricted
zz Is caused by pathologies involving the superior oblique
MOVEMENTS
(SO) tendon-trochlea complex that restricts the passage
SECONDARY Equal to primary More than and movement of SO through the trochlea.
DEVIATION primary
DIPLOPIA Absent Present Causes
FALSE PROJECTION Absent Present zz Congenital, trauma, Rheumatoid arthritis.
ABNORMAL HEAD Absent Usually present
POSTURE
Duane’s Retraction Syndrome
VERTIGO Absent Present
zz Characterized by co-contraction of the medial and
lateral rectus muscles due to anomalous innervation
of one of the extraocular muscle antagonists during
HYPERMETROPIA# embryogenesis.
Patient accommodates to see near vision → More convergence zz Abducens nerve fails to innervate Lateral rectus. So
→ ESOTROPIA the Oculomotor nerve supplies both lateral rectus and
zz In Apparent/Pseudosquint, EXOTROPIA occurs in hyper- medial retcus.
metropia.
MYOPIA# Types
Convergence is weak since it is not used much; eyes tend to zz Type I: Limited abduction with or without esotropia - is
deviate out → EXOTROPIA more common
zz In Apparent squint, ESOTROPIA occurs zz Type II: Limited adduction with or without exotropia
zz Type III: Limitation of both abduction and adduction
Management of Strabismus and any form of horizontal strabismus

zz 4 Os
 O – OPTICAL CORRECTION - Prism
 O – OCCLUSION THERAPY – for amblyopia NYSTAGMUS
 O – ORTHOPTIC EXERCISES – for amblyopia zz Involuntary, oscillatory, purposeless to and fro
 O – OPERTAIVE MEASURES movements.

Types
zz Physiological (End point, OKN and vestibular)
zz Pathological: Congenital and acquired.
OPHTHALMOLOGY

Fig. 13: RAF ruler – to measure Near point of Convergence and

Accomodation
 Chapter 22  |  Neuro-ophthalmology and Squint

TABLE 15: Clinical features of various types of Nystagmus

PHYSIOLOGICAL NYSTAGMUS
487
Types Clinical Features Site of Lesion
End Point Nystagmus • Fine jerky nystagmus in extremes of gaze.
• The fast phase is on the direction of gaze.
Optokinetic Nystagmus • Jerky nystagmus induced by moving repetitive • Parieto-occipito temporal region control the slow
targets (pursuit) phase.
• Frontal lobe control the rapid saccadic phase
Vestibular Nystagmus • Destructive lesions induce nystagmus to the • Altered input from the vestibular nuclei to
opposite side# horizontal gaze centers.
• Irritative lesions produce fast phase in the same
direction#
• CALORIE TEST
COWS
Syringing Cold water in one ear → Opposite side
nystagmus
Warm water → Same side nystagmus
CUWD
Syringing Cold water in both ears → Upward gaze
Syringing Warm water in both ears → Downward gaze
PATHOLOGICAL NYSTAGMUS
CONGENITAL • Pendular type nystagmus#
• Dampened by convergence and not present during
sleep
• There is usually null point- position of gaze in which
nystagmus minimal and compensatory head posture
may develop.
UPBEAT NYSTAGMUS • The vermis of cerebellum or the brainstem#
• Phenytoin toxicity #
DOWN BEAT • Posterior fossa lesion at the level of Foramen
NYSTAGMUS Magnum#
REBOUND • Changes the direction of the nystagmus with • Cerebellar lesion#
NYSTAGMUS sustained gaze
SEA –SAW • One eye elevates and intorts and other eye • The Chiasma or the Third ventricle #
NYSTAGMUS depresses and extorts
BRUNS NYSTAGMUS • Coarse horizontal nystagmus in one eye and fine • Cerebellopontine angle tumors# eg., Acoustic
high frequency vestibular nystagmus in other eye neuroma.
GAZE EVOKED • No nystagmus in the primary position, it appears • Alcohol intoxication, Barbiturates, Cerebellar and
NYSTAGMUS when the eyes look to the side. brain stem lesion
ATAXIC NYSTAGMUS • Internuclear Ophthalmoplegia
MINER’ S NYSTAGMUS • Rotatory nystagmus
OPHTHALMOLOGY

• Syndromes associated with abducent nerve palsy are:

ƒƒ Foville: Involves dorsal pons, characterized by ipsilateral involvement of cranial nerves 5,6,7, and 8, Horner syndrome
and horizontal gaze palsy.
ƒƒ Millard-Gubler: Lesion at the level of pyramidal tract, ipsilateral abducent palsy, LMN facial palsy and contralateral
hemiplegia.
 Triple O

488
Image-Based Questions
1. Type of optic atrophy seen in the condition shown 3. Identify the type of pupil, if lesion occurs at the
below is level as shown in this picture

A. Primary
B. Secondary
C. Consecutive A. Marcus gunn pupil
D. Cavernous B. Wernicke Hemianopic pupil
C. Hutchinson pupil
D. Argyl Robertson pupil

2. Identify the typical field changes associated with

the condition shown in this picture
4.  Type of therapy shown in the picture is used to treat

A. Binasal hemianopia
B. Bitemporal hemianopia
C. Upper quadrantanopia
OPHTHALMOLOGY

D. Lower quadrantanopia
A. Myopia B. Anisometropia
C. Amblyopia D. Hypermetropia
 Chapter 22  |  Neuro-ophthalmology and Squint

5. 60 years old male with neurological symptoms and 8.  Instrument shown below is used for
his field reports as shown in the picture. Where is the 489
site of lesion

A. Temporal lobe B. Parietal lobe

C. Occipital cortex D. Frontal lobe

A. Optic nerve function B. Corneal transparency

6. Identify the type of field defect usually associated
C. Macular function D. Visual acquity
with the condition shown below

9.  Longest portion of the structure shown below is

A. Bitemporal
B. Enlargement of blind spot
C. Arcuate scotoma A. Intraocular B. Intraorbital
D. Bjerum scotoma C. Canalicular D. Intracranial

7.  Elevators of the Eye ball is

10.  The chart shown below is used to assess

OPHTHALMOLOGY

A. 1and 3 B. 1and 2
C. 2 and 4 D. 1and 4 A. Macular function B. Color vision
C. Binocular vision D. Diplopia
 Triple O

11.  Evaluate the below picture and come to a diagnosis.

490

A. Internuclear ophthalmoplegia B. Left 3rd nerve palsy

C. Horizontal gaze palsy D. Duane retraction syndrome

Answers of Image-Based Questions

1. C. consecutive (Parson’s, 22nd edition, P.No.362)
Fundus photograph suggestive of Retinitis pigmentosa and pallor of the disc (waxy pallor). Type of optic atrophy in this condition
is consecutive optic atrophy.
2. B. Bitemporal hemianopia (AK Khurana’s, 7th edition, P.No.325)
MRI picture shows tumor of the pituitary gland. Pituitary gland tumor compress on the optic chiasm and produce classically
bitemporal hemianopia.
3. B. Wernicke hermianopic pupil (AK Khurana’s, 7th edition, P.No.328)
Site shown in this picture is optic tract, it contains fibers of same side temporal retina and opposite side nasal retina. So lesion
at this level cause wernicke hemianopic pupil.
4. C. Amblyopia (Parson’s, 22nd edition, P.No.422)
Clinical photograph shows occlusion of the left eye. Occlusion of normal eye is done as a therapy for amblyopia.
5. B. Parietal lube (Parson’s, 22nd edition, P.No.529)
Field defect shown in this picture is inferior quadrantonopia. It is seen in lesion of parietal lobe. Superior quadrantonopia is
seen in temporal lobe lesion.
6. B. Enlargement of blind spot (Kanski,’s 7th edition, P.No.802)
Fundus photo suggestive of papilledema. Enlargement of blind spot usually is seen in Blind spot in the field is corresponds to
area of optic disc.
7. D. 1 and 4 (Parson’s, 22nd edition, P.No.405)
Elevators of the eyes are superior rectus and Inferior oblique.
OPHTHALMOLOGY

8. C. Macular function (Parson’s, 22nd edition, P.No.423)

Instrument shown in this picture is Maddox rod. It is composed of serially arranged cylindrical lenses. It is used to test the
macular function.
9. B. Intraorbital (Parson’s, 22nd edition, P.No.348)
Shortest portion of the optic nerve is intraocular (1mm) and longest portion of the optic nerve is intra orbital (25-30mm)
10. D. Diplopia (AK Khurana’s, 7th edition, P.No.357)
In diplopia chart maximum separation of image will be on the side of muscle action.
11. A. Internuclear ophthalmoplegia (Kanski’s,8th edition, P.No. 831)
In unilateral internuclear ophthalmoplegia restriction of adduction on the same side and Nystagmus on the opposite eye.
 Chapter 22  |  Neuro-ophthalmology and Squint

491
Multiple Choice Questions
1. Clinical Diagnosis for the given pic? (AIIMS Dec 2018) 6. This test is used in (Recent pattern 2019 )

A. Internuclear ophthalmoplegia
B. Third nerve palsy
C. WEBINO
d. Congenital ptosis
2. A 36 year old female patient complains of recurrent
episodes of diminution of vision in both eyes. She
was treated with steroids after which her symptoms
improved. On examination, vision in RE – 6/60 and LE
– 6/18 and Colour vision is defective in both eyes. She
also develops spastic paraplegia. What is the diagnosis?
 (AIIMS Dec 2018)
A. Multiple sclerosis
B. Neuromyelitis optica
C. Carotid artery dissection
D. Syringomyelia
3. The center for vertical gaze is: (AIIMS Dec 2018)
A. Pontine Paramedian Reticular Formation
B. Raphae nucleus
C. Rostreal interstitial nucleus of Cajal
D. Nucleus of Perlia
4. When a small target is oscillated in front of a patient
with binocular vision, patient sees movement of the
object in elliptical orbit rather than to & fro path. What
is this phenomena? (JIPER May 2018)
A. Oppenheime
B. Pulfrich phenomenon
C. Uthoff phenomenon
D. Paroxysmal Convergence spasm phenomenon
5. Esotropia is commonly seen in which type of refractive
error? (Recent pattern 2019)
A. Myopia B. Hypermetropia
C. Astigmatism D. Presbyopia
A. Squint B. Heterophoria
C. Esotropia D. All the above
7. All are true about optic nerve except  (PGI May 10)
A. Arises from axons of bipolar neurons
B. 4cm long
C. Covered by 3 layers continuous with meninges
D. Crossed by ophthalmic artery
8. Swinging light test is positive in
 (Recent pattern 2015-16)
A. Conjunctivitis B. Glaucoma
C. Retrobulbar neuritis D. Keratoconus
9. All of the following statements about Argyll Robertson
OPHTHALMOLOGY
Pupil are correct, Except:  (AI 11)
A. Near Reflex Normal
B. Direct light Reflex Absent
C. Consensual Light Reflex Normal
D. Visual Acuity is Normal
10. Lesion of right optic tract will lead to:
 (Recent pattern 2014-15)
A. Bitemporal hemianopia
B. Right homonymus hemianopia
C. Left homonymous hemianopia
D. Binasal hemianopia
 Triple O

11. Bitemporal hemianopic field defect is characteristics of 20. Unilateral sudden complete loss of vision (Amaurosis
492  (Recent pattern 2014-15, AIIMS May 06) fugax) is due to lesion in (JIPMER 11)
A. Glaucoma B. Optic neuritis A. Internal carotid artery B. Middle cerebral artery
C. Pituitary tumour D. Retinal detachment C. Anterior cerebral artery D. Basilar artery
12. Visual field defect in pituitary tumor with supracellar 21. Amaurosis fugax is due to (Recent pattern 2015-16)
extension is  (Jipmer 11) A. TIA B. Tobacco
A. Bitemporal hemianopia C. Optic neuritis D. Papilloedema
B. Binasal hemianopia 22. Dilator pupillae is supplied by (AIIMS, Nov 11)
C. Homonymous hemianopia A. Post-ganglionic parasympathetic fibers from Edinger
D. Pie in the sky vision Westphal nucleus
13. Unilateral Papilloedema with optic atrophy on the other B. Post-ganglionic sympathetic fibers from cervical
side is a feature of  (2015-17) sympathetic chain
A. Foster kennedy syndrome C. IIIrd nerve
B. Fisher syndrome D. Sympathetic fibers from fronto-orbital branch of V
C. Vogt-koyanagi harada syndrome nerve
D. WAGR syndrome 23. Longest and thinnest extraocular muscle is
14. Vitamin B12 deficiency is likely to cause  (2013-14)
A. Bitemporal heminanopia (2014-15) A. SR B. IR
B. Binasal hemianopia C. SO D. IO
C. Heteronymous hemianopia 24. Distance of medial rectus from limbus is
D. Centrocecal scotoma  (Recent pattern 15)
15. Fundoscopy of a patient shows chalky white optic A. 4.5mm B. 5.5mm
disc with well defined margins. Retinal vessels and C. 7.0mm D. 10mm
surrounding Retina appears normal. Which of the 25. Action of superior oblique muscle is/are (PGI Nov 09)
following is the most likely diagnosis (AI 12) A. Extorsion B. Abduction
A. Primary Optic Atrophy C. Intorsion D. Depression
B. Post-neuritis secondary optic atrophy E. Elevator
C. Glaucomatous optic atrophy 26. Downward and lateral gaze is action of 
D. Consecutive optic atrophy  (Recent pattern 2016)
16. A young man with blurring of vision in right eye, A. Inferior oblique B. Medial rectus
followed by left eye after 3 months, showing disc C. Superior oblique D. Lateral rectus
hyperemia, edema, circumpapillary telangiectasia with 27. Elevators of eye is (Recent pattern 2015)
normal papillary response with centrocecal scotoma on A. SR and IO B. IO and SO
perimetry, the cause is  (AIIMS, May 09) C. IR and S D. SO SR
A. Typical optic neuritis 28. Yolk muscle pair is  (Recent pattern 2012-13)
B. Acute Papilledema A. Rt MR and Rt LR
C. Toxic optic neuropathy B. Rt MR and Lt LR
D. Leber’s hereditary optic neuropathy C. Rt SO and Lt IO
17. Regarding color blindness true is (PGI Dec 07) D. Rt SR and LT SR
A. Mainly congenital 29. All are characteristics of 3rd nerve except
B. Can be tested with Fansworth 100 hue test A. Carries parasympathetic nerve
C. Isihara chart test red/green color blindness B. Supplies inferior oblique
D. Jerlin-Merlin cotton wool tests it C. Enters orbit through the inferior orbital fissure
18. Anisocoria in dim light is maximally seen in  D. Causes miosis
A. 3rd nerve palsy (2014-15) 30. All are seen in 3rd nerve palsy except (AIIMS, Nov 11)
B. Pharmacological mydriasis A. Ptosis
OPHTHALMOLOGY

C. Horner syndrome B. Diplopia

D. Parasympathetic paralysis C. Miosis
19. In case of anisocoria when 1% pilocarpine is instilled D. Outward deviation of eye
into the eye with abnormally dilated pupil, pupil 31. Left sided sixth nerve palsy would lead to 
remains dilated. Cause of anisocoria may be:  (AIIMS, Nov 08)
A. Adies pupil (AIIMS, Nov 11) A. Accommodation paresis of left eye
B. Pharmacological blockage B. Ptosis of left eye
C. Uncal herniation C. Adduction weakness of left eye
D. Diabetic III cranial nerve palsy D. Diplopia in left gaze
 Chapter 22  |  Neuro-ophthalmology and Squint

32. A patient presented with his head tilted towards right. 41. What is the most common cause of amblyopia?
On examination, he was having left Hypertropia which A. Squint B. Tobacco 493
is increased on looking towards right or medially. The C. Methyl alcohol D. Hypermetropia
muscle which is most likely paralyzed is 42. Pupillary reflex pathway-All of the following are a part
A. Left superior oblique (AIIMS, May 08) except–
B. Left inferior oblique A. Edinger westphal nucleus
C. Right superior oblique B. Pretectal nucles
D. Right inferior oblique C. Medial geniculate body
33. The reciprocal inhibition of antagonist muscle upon D. Retinal ganglion cell
lateral gaze is explained by (AIIMS, May 08) 43. Anisokonia is?
A. Sherrington’s law B. Hering’s law A. Projection of different colored images into visual cortex
C. Laplace law D. Hick’s law B. Projection of different shaped images into visual cortex
34. Child with mild squint. Intrauterine, birth history, of two retinae
developmental history till date all normal. Corneal C. Change in the velocity of perceived objects
reflex normal. All other eye parameters normal except D. Partial intermittent visual loss
exaggerated epicanthal fold. Diagnosis is 44. Ophthalmic finding of acute meningococcal meningitis
 (Recent pattern 2013-14) are all except:
A. Pseudostrabismus B. Accommodative squint A. Ocular motility palsy
C. Exophoria D. Esophoria B. Pailloedema
35. In concomitant squint (Recent pattern 2013-14) C. Optic neuritis
A. Primary deviation > Secondary deviation D. Glaucoma
B. Secondary deviation > Primary deviation 45. Optic glioma is associated with
C. Primary deviation = Secondary deviation A. Neurofibromatosis 1
D. None B. Neurofibromatosis 2
36. Angle of squint is measured by(Recent pattern 2015-16) C. Sturge weber syndrome
A. Gonioscopy D. Von hipple landau syndrome
B. Prism 46. Macular sparing is seen in lesion of–
C. Retinoscopy A. Visual cortex
D. Keratometry B. Optical tract
37. A 26 years old male with restriction of eye movements in C. Optical chiasma
all directions and moderate ptosis but with no Diplopia D. Optic nerve
or squint. Diagnosis is  (AIIMS, Nov 09) 47. Exotropia occurs due to–
A. Thyroid ophthalmopathy A. Third nerve palsy
B. Chronic progressive external ophthalmoplegia B. Optic neuritis
C. Myasthenia gravis C. Abducens
D. Multiple cranial nerve palsies D. Papilloedema
38. Ophthalmoplegic migraine means (AI 11) 48. Vitamin deficiency causing optic atrophy is
A. Headache with irreversible loss of ophthalmic nerve A. Vitamin A
function B. Vitamin B
B. Recurrent transient 3rd nerve palsy associated with C. Vitamin C
headache D. Vitamin D
C. Headache associated with 3rd, 4th and 6th nerve palsy 49. Paralystic squint is–
D. Headache associated with optic neuritis A. Incomitant squint
39. Final center for horizontal movements of eye is  (AI 08) B. Exophoria
A. Abducent nucleus C. Estropia
B. Trochlear nucleus D. Heterotropia
OPHTHALMOLOGY

C. Oculomotor nucleus
D. Vestibular nucleus
40. Weakness of both Adduction and Abduction is seen in
 (AIIMS, May 12)
A. Duane’s Retraction Syndrome Type 1
B. Duane’s Retraction syndrome Type 2
C. Duane’s Retraction syndrome Type 3
D. All
50. All the following signs could result from infection within
the right cavernous sinus except–
A. Constricted pupil in response to light
B. Engorgement of the retinal veins upon ophthalmoscopic
examination
C. Ptosis of the right eyelid
D. Right ophthalmoplegia
 Triple O
494 Answers with Explanations
1. B. Third cranial nerve palsy  (Parson’s Diseases of the eye,
 22nd edition, page no. 443)
2. B. Neuromyelitis Optica  (A K Khurana Comprehensive
 Ophthalmology , 6th edition, pg no. 317)
3. C. Rostreal interstitial nucleus of Cajal(Parson’s Diseases
 of the eye, 22nd edition, page no. 410)
4. B. Pulfrich phenomenon (Albert & Jacobie ‘s Principles
and practice of ophthalmology, 3rd edition, page no. 3874)
5. A. Hypermetropia
 (Parson’s Diseases of the eye , 22nd edition, page no. 246.)
6. D. All the above  (Parson’s
 Diseases of the eye, 22nd edition, page no. 184; A
 K Khurana Comprehensive Ophthalmology, 7th edition,
 pg no. 365)
7. A. Arises from axons of bipolar neurons, B. 4cm long
 (AK Khurana’s, 7th edition, P.No. 323)
Optic nerve consists of approximately 1.2million axons
that arises from the retinal ganglion cells. Optic nerve has
no power regeneration.
8. C. Retrobulbar neuritis
 (AK Khurana’s, 7th edition, P.No. 330)
Swinging flash light test is used to cheek the RAPD.
9. C. Consensual Light Reflex Normal 
 (AK Khurana’s, 7th edition, P.No. 328)
Argyl Robertson pupil (Remember as ARP- accommodation
[i.e. near reflex] reflex is present; PRA: Pupillary Reflex
absent). Lesion is in tectum and invariable syphilitic origin.
10. C. Left homonymous hemianopia 
 (Parson’s, 22nd edition, P.No.34)
Right optic tract contains fibers from right eye temporal
retina and left eye nasal retina. So it causes left sided
homonymous hemianopia.
11. C. Pituitary tumour  (Parson’s, 22nd edition, P.No.34)
Pituitary gland tumors compress the central portion of
optic chiasma, where the nasal fibers from both sides
decussate and produce bitemporal hemianopia.
12. A. Bitemporal hemianopia 
 (Parson’s, 22nd edition, P.No.34)
OPHTHALMOLOGY
13. A. Foster kennedy syndrome 
 (Parson’s, 22nd edition, P.No.353)
Foster kennedy syndrome is mainly seen in olfactory
groove Meningioma or tumors of the orbital surface of
frontal lobe.
14. D. Centrocecal scotoma 
 (Parson’s, 22nd edition, P.No.349, 350)
Vitamin B12 deficiency causes nutritional optic neuropathy
and produce centrocecal scotoma.
15. A. Primary optic atrophy 
 (Parson’s, 22nd edition, P.No.362, 363)
Chalky white optic disc, well defined disc margin, normal
cup disc ratio, normal blood vessels and retina are the
features of primary optic atrophy.
16. D. Leber’s hereditary optic neuropathy 
 (Parson’s, 22nd edition, P.No.368)
Leber’s hereditary optic neuropathy is usually present
with hyperaemic disc, (in early life) and associated with
telengiectasia is also seen.
17. A. Mainly congenital, B. Can be tested with Fansworth
100 hue test, C. Isihara chart test red/green color
blindness (Parson’s, 22nd edition, P.No.108,109)
18. C. Horner syndrome 
 (AK Khurana’s, 7th edition, P.No.317)
Features of Horner syndrome include miosis, enoph-
thalmos, mild ptosis, anhydrosis and loss of ciliospinal
reflex.
19. B. Pharmacological blockage 
 (AK Khurana’s, 7th edition, P.No.329)
Pupil dilated due to neurological cause will constrict with
pilocarpine and due to cycloplegics (mydriatrics) will not
constrict with pilocarpine.
20. A. Internal carotid artery 
 (AK Khurana’s, 7th edition, P.No.343)
Ophthalmic artery is a branch of internal carotid artery. So
any occlusion in the internal carotid artery leads to sudden
loss of vision.
21. A. TIA  (AK Khurana’s, 7th edition, P.No.343)
Transient decrease in blood supply to the eye or optic
nerve is called amaurosis fugax. Emboli from the carotid
artery is the most common cause for it.
22. B. Post-ganglionic sympathetic fibers from cervical
sympathetic chain  (AK Khurana’s, 7th edition, P.No.152)
Post ganglionic sympathetic fibers from superior cervical
ganglion supplies the dilator pupillae.
23. C. SO  (Ref, Parson’s, 22nd edition, P.No.404)
Superior oblique primary action is intersion other actions
are abduction and depression.
24. B. 5.5mm  (Parson’s, 22nd edition, P.No.403)
Medical retus is closest muscle to the limbus. It may be
damaged while doing Nasal pterygium.
25. C. Intorsion  (Parson’s, 22nd edition, P.No.405)
26. C. Superior oblique  (Parson’s, 22nd edition, P.No.405)
Superior oblique is supplied by Trochlear nerve.

27. A. SR and IO  (Parson’s, 22nd edition, P.No.405)
Superior rectus muscle and inferior oblique is supplied
by oculomotor nerve. Paralysis of these muscle causes
diplopia on upgaze.
28. B. Rt MR and Lt LR  (Parson’s, 22nd edition, P.No.407)
Yoke muscles are pair of muscle (one muscle from each
eye) which moves the eyeball in same direction (conjugate
eye movements).
29. C. Enters orbit through the inferior orbital fissure
 (Parson’s, 22nd edition, P.No.408)
Oculomotor nerves enter the orbit through superior orbital
fissure.
30. C. Miosis  (Parson’s, 22nd edition, P.No.442)
Ptosis is due to LPS muscle palsy, diplopia is due to
paralysis of extraocular muscles, outward deviation of eye
ball is due to unopposed action of lateral rectus pupil is
usually dilated.
31. D. Diplopia in left gaze  (Parson’s, 22nd edition, P.No.440)
Diplopia in muscle palsy, maximum separation of image is
on the side of gaze (muscle action)
32. A. Left superior oblique (Parson’s, 22nd edition, P.No.441)
Superior oblique action is depression. If superior oblique is
paralysed, it cause hypertrophia on the same side. Superior
oblique is supplied by Trochlear nerve.
33. A. Sherrington’s law  (Parson’s, 22nd edition, P.No.408)
Hering’s law is equal simultaneous innervations of yoke
muscle.
34. A. Pseudostrabismus  (Parson’s, 22nd edition, P.No.470)
Epicanthal fold (Extra fold of skin extending from upperlid
to lower lid on the medical side) results appearance of
Esotropia is called pseudostrabismus in this condition.
35. C. Primary deviation = Secondary deviation
 (Parson’s, 22nd edition, P.No.416)
36. B. Prism  (Parson’s, 22nd edition, P.No.421)
2∆ (Prism) deviation is equal to 1° (degree)
37. B. Chronic progressive external ophthalmoplegia
 (Kanski 7th edition P.No.852)
Restriction of ocular movement in both eyes are symmetrical
in chronic progressive external ophthalmoplegia, so there
is no diplopia.
38. B. Recurrent transient 3rd nerve palsy associated with
headache (Parson’s 22nd edition P.No 520)
Ipsilateral paralysis of one or more extraocular muscles as
migranous headache is resolving. This is more common in
childhood.
39. A. Abducent nucleus  (Parson’s 22nd edition P.No 410)
The center controlling horizontal conjugate movements
near the sixth nucleus (abducent nucleus) is the pontine
Chapter 22  |  Neuro-ophthalmology and Squint
paramedian reticular formation (PPRF); It controls
conjugate horizontal movement to the Ipsilateral side. 495
40. C. Duane’s Retraction Syndrome Type 3 
 (Khanski’s, 7th edtion, P.No.773)
Type I: (most common type) limited abduction and normal
adduction.
Type II (least common): Limited adduction and normal
abduction.
Type III : Limited adduction and abduction.
41. A. Squint (Kanski’s 8th edition, P.No.737)
Strabismic amblyopia results from abnormal binocular
inter action with continue monocular suppression of the
deviating eye.
42. C. Medial geniculate body
 (Parson’s 22nd edition, P.No33, 34)
Pupillary reflex involves from retina to optic nerve – optic
chaisma – optic tract – pretectal nucles – edinger westphal
nucleus- parasympathetic fibres through 3rd nerve – cilliary
ganglion – short cilliary nerves – sphincter pupillae.
43. B. Projection of different shaped images into visual
cortex of two retinae (Parson’s 22nd edition, P.No.78)
Difference in the size of retinal images of the two eyes is
called Ansiokonia.
44. D. Glaucoma (Parson’s 22nd edition, P.No. 522,523)
Glaucoma is not related to meningitis. Bilateral 3rd nerve
paralysis can occur in syphilitic basal meningitis.
45. A. Neurofibromatosis 1 (Kanski’s 8th edition, P.No.845)
Optic nerve Glioma, Meningioma, lisch nodules, prominent
corneal nerves and plexiform neurofibromatosis are
the common ocular features of NF-1. Glaucoma is not a
common association.
46. A. Visual cortex (Parson’s 22nd edition, P.No. 507)
Macular sparing is due to bilateral innervation and dual
blood supply.
47. A. Third nerve palsy (Parson’s 22nd edition, P.No. 442)
In isolated 3rd nerve palsy lateral rectus and superior
oblique muscles are not affected. So the eyeball will be in
abducted position.
48. B. Vitamin B (Parson’s 22nd edition, P.No.365)
Vitamin B12 and folic acid deficiency is associated with
optic atrophy.
49. A. Incomitant squint (Parson’s 22nd edition, P.No.434)
It is associated with diplopia, restriction of extraocular
OPHTHALMOLOGY
movements and abnormal head posture.
50. A. Constricted pupil in response to light
 (Parson’s 22nd edition, P.No.490)
6th nerve is first affected in cavernous sinus thrombosis.