NEURO-OPHTHALMOLOGY

Bethlehem Girma(M.D)
Assistant professor of ophthalmology

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 Neuro-ophthalmologic Disorders
• Clinical evaluation of NO patients
• Pupillary abnormalities
• Visual field defects
• Optic neuritis
• Papilloedema
• Optic nerve atrophy
• Disorders of cranial nerves
• Nystagmus

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 Clinical Evaluation
History
 Identification: Name, age, sex, occupation
 Chief complaint
• Document in pt’s own words
• Note the duration & lateralization or binocularity of
visual deficits
 History of present illness(HPI)
• Document the onset, quality, severity, lateralization,
and associated features of pt’s visual symptoms.

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Vital diagnostic elements:
Lateralization, location, duration, degree of
recovery, presence or absence of pain
Determine if pt’s visual loss is monocular or bilateral
If visual loss is monocular, ascertain if horizontal
respect to visual loss is present
If visual loss is bilateral, exclude a homonymous or
heteronymous defect
Document if perceived vision loss is at distance fixation,
near fixation or both

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. . . Vital diagnostic elements
Degree of recovery
The degree of recovery and the time until recovery after visual
loss are other important elements.
Transient visual obscurations that last for seconds may be
due to increased intracranial pressure.
Painless unilateral visual loss that lasts several minutes may
be embolic (amaurosis fugax), especially if it respects the
horizontal midline
Gaze-evoked amaurosis may be due to a meningioma of the
optic nerve sheath or another tumor of the orbital apex.

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Transient visual loss with scintillations and
movement that lasts 20-30 minutes then completely
resolves suggests migraine.
Sudden-onset visual loss with little recovery
suggests an ischemic event.
Sub acute visual loss with almost complete
recovery of vision after several weeks or months is
characteristic of optic neuritis.
Slowly progressive visual loss may be due to a
compressive optic neuropathy.
Visual loss with headache in an elderly patient may
be due to giant cell arteritis.

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 Reading-related visual loss in older patients
When older patients complain of visual loss when
reading, presbyopia is not always the cause.
Determine if the patient has difficulty seeing
whole words, finding the next line (possible left
homonymous field loss), or reading the next word
(possible right homonymous field loss).

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 Diplopia
Diplopia is a common neuro-ophthalmic complaint.
Document the following features:
-monocular or binocular nature,
-is the double vision horizontal, vertical or oblique?,
-is the double vision the same in all positions of gaze
(comitant) or varies with gaze direction(incomitant)?
-is the diplopia intermittent, variable or constant?
-associated ptosis or known dysthyroidism.
Double vision that persists despite closing either eye
(monocular) but improves with pinhole suggests
1.monocular diplopia from uncorrected refractive error,
2.optical aberrations of the ocular media, or
3.on occasion retinal disorders that distort the fovea

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. . . Diplopia
 Monocular diplopia that improves with blinking often is due to
the irregular astigmatism from dry eyes.
 Common causes of binocular diplopia are paralytic squint,
myasthenia gravis, DM, thyroid disorders, blow-out fracture of
floor of the orbit
 Binocular diplopia with vertical or diagonal separation of objects,
worse when the patient is reading or climbing stairs and better
with head tilt to 1 side, suggests palsy of cranial nerve (CN) IV.
 Binocular diplopia with horizontal separation that is worse in the
distance suggests CN VI palsy.
 Patients who develop complete CN III palsy initially may complain
of diplopia. They later indicate that the diplopia resolves, because
ptosis occludes the visual axis.
 Variability or worsening of the diplopia as the day progresses
with associated ptosis suggests myasthenia gravis.
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 Periocular pain and headache
• Always consider giant cell arteritis in older patients who
present with headache, scalp tenderness, or occipital
tenderness.
• Burning discomfort in a unilateral segment of the trigeminal
nerve distribution occasionally precedes the appearance of
herpetic vesicles; at times, early herpes zoster can be mistaken
for temporal arteritis.
• Migraine headaches, which often consist of several hours of
throbbing hemicranial and retro-orbital discomfort, are
common.
Migraine may be accompanied by visual scintillations, which
resolve over 15-30 minutes. A family history of migraine is
common, and trigger factors, such as certain foods, may be
noted. 10
Eye pain has several differential diagnoses
– Dry eye, Corneal abrasions, iritis, and angle-
closure glaucoma
Angle-closure glaucoma may be misdiagnosed as an
intracranial process (e.g., aneurysm) because of the
prominent headache, nausea and vomiting, and
Mid dilated pupil.

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. . . CLINICAL EVALUATION
Examination
Refraction
Visual acuity
Color vision test
Amsler grid test
Visual field exam
 confrontation field testing
 tangent screening
 Goldmann perimetry
 automated perimetry

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. . .Examination
 Pupils
 Record the size , difference in size (Anisocoria)
 Pupillary light reflex
 RAPD (or Marcus Gunn pupil)
 Near response
• Anterior segment exam
• Fundoscopy
Dilated fundus examination
 Papilloedema
 Optic atrophy
 Retina findings

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. . . 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

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• Eyelids
– Lid retraction
– Ptosis
• Orbit
– Proptosis
• Extra ocular examination
– BP in Papilloedema
– Neurologic exam

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 Neuro-ophthalmologic Disorders
• Clinical evaluation of NO patients
• Pupillary abnormalities
• Visual field defects
• Optic neuritis
• Papilloedema
• Optic nerve atrophy
• Disorders of cranial nerves
• Nystagmus
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PUPILLARY ABNORMALITIES

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 Applied Anatomy
 The three major optical functions of pupil are:
-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.

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 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 from retinal ganglion cells to the
pretectal area;
2.An intercalated neuron from the pretectal complex to
the parasympathetic motor pool (Edinger–Westphal
nucleus) of the oculomotor nuclear complex and
3.The efferent parasympathetic outflow with the
oculomotor nerve to the ciliary ganglion; and from
there to the pupillary sphincter.

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20
 NEAR REFLEX
Is a synkinesis rather than a true reflex consisting of three
components
1. Constriction of pupil – Miosis
2. Increased tone of ciliary muscle – Accommodation
3. Contraction of medial rectus - Convergence
Major stimulus to the reflex is blurring of visual objects coming
closer to the near point of the eye.
The final pathways for the near and light reflexes are identical
(i. e. third nerve  ciliary ganglion short ciliary nerves)but the
centre for the near reflex is ill-defined.
 Vision is not a prerequisite for the near reflex and there is no clinical
condition in which the light reflex is present but the near
response is absent.

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 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.

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 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.
• RAPD (Relative afferent pupillary defect)
- RAPD (Marcus Gunn pupil) is caused by an incomplete optic
nerve lesion or severe retinal disease.
-The pupils respond weakly to stimulation of the diseased eye
and briskly to that of the normal eye.

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The swinging flash light test where a light source is alternatively
switched from one eye to the other and back thus stimulating
each eye in rapid succession.
 When the normal eye is stimulated it results in brisk
constriction of both pupils.
 When the light is swung to the diseased eye, both pupils
dilate instead of constricting.
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 l eye.
 Relative afferent pupillary defects do not cause anisocoria
(pupillary inequality) in humans, because any changes in light
input are distributed equally to both pupils.

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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

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 Efferent Pupillary Defects
• These are seen in disorders that interfere with the contraction
or dilatation of the pupil.
• The location of damage affecting the pupillary response to
light, dark, or near may be in
– the midbrain
– along the course of the peripheral nerves supplying the iris dilator or
sphincter muscle, or
– directly in the iris tissue itself.
• Most diseases causing efferent pupillary defects are unilateral
or asymmetric and therefore cause unequal pupils (anisocoria)

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• To sort out which muscle is not working
properly, it helps to know how the anisocoria
is influenced by light.
• Greater anisocoria in the light reflects damage
to the parasympathetic pathway
• Anisocoria that is worse in the dark reflects
damage to the sympathetic pathway.

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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

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 Physiologic Anisocoria
• A normal, asymmetric contribution of the
sympathetic and parasympathetic nervous
systems to each pupil, regardless of ambient
lighting.
• Pupil size disparity is the same in light as in
dark, and the pupils react normally to light.
• In 10% of otherwise healthy individuals, the
difference in pupil size is 1 mm or less.

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 HORNER’S SYNDROME( oculo sympathetic defect)
Symptoms: Droopy eyelid, pupil size disparity; often asymptomatic
Signs:
-Mild ptosis(1-2mm) due to paresis of Muller's muscle
-Miosis  due to the unopposed action of sphincter pupillae
- Anhydrosis(loss of sweating ability)  reduced ipsilateral sweating but
only if the lesion is below the superior cervical gang lion because the
fibers supplying the skin of the face run a long the external carotid artery.
– Pseudo-enophthalmos ( b/c of ptosis & lower lid elevation)
– Ocular hypotony
– Conjunctival congestion(transient dilated conjunctival & facial vessels)
due to the denervation vasoconstrictor fibers
– Iris heterochromia (if congenital)
– Paradoxical contra lateral eyelid retraction
– Increased amplitude of accommodation

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Rt Horner’s syndrome

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. . . HORNER’S SYNDROME
• Etiology
– First order neuron disorder: stroke, tumor
– Second order neuron disorder: tumor(e.g. lung
carcinoma)=>pan coast'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)

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. . . HORNER’S SYNDROME
Work-up
– Diagnosis confirmed with a cocaine test(10%)
– If the sympathetic discharge is impaired, less nor epinephrine is
released , resulting in less mydriasis compared with the normal eye.

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. . . HORNER’S SYNDROME
• Hydroxyamphetamine 1% is used to distinguish a 3rd – order neuron
disorder from 1st or 2nd –order neuron disorder.
• Failure of the Horner’s pupil to dilate to an equivalent degree as the
fellow eye indicates a 3rd –order neuron lesion.

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 ADIE’S TONIC PUPIL
Adie (tonic) pupil is caused by denervation of the postganglionic
supply to the sphincter pupillae and the ciliary muscle.
It typically affects young adults and is unilateral l in 80% of cases.
Etiology
– Idiopathic, orbital trauma or infection, herpes
zoster infection, diabetes, autonomic
neuropathies, others
Symptoms:
– Difference in the size of the pupils, blurred vision; may be
asymptomatic

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 . . . ADIE’S TONIC PUPIL
Signs:
– An irregularly dilated pupil exhibiting minimal or no
reaction to light, slow constriction to convergence ,
and slow redilatation.
– The pupil demonstrates super sensitivity to weak
cholinergic agents(e.g. pilocarpine 0.125%)
– It may develop acutely and may become bilateral
– The pupil dilates normally to mydriatic agents.
– Deep tendon reflexes (knee and ankle) are often
absent( Adie’s syndrome)
– The involved pupil may become smaller than the
normal pupil over time.
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. . . ADIE’S TONIC PUPIL
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 contra lateral pupil in Adie’s syndrome.

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 ARGYLL ROBERTSON PUPIL

• This is caused by neurosyphilis and is characterized by

bilateral but asymmetric involvement where the pupil
are small and irregular.
Symptom: usually asymptomatic
Signs:
– Small, irregular pupil that reacts poorly or not at all to light
but constricts normally during convergence. By definition,
vision is normal
– The pupil does not dilate well.

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. . . ARGYLL ROBERTSON PUPIL
• Work-up
– Test the pupillary reaction to light and convergence
– Slit-lamp exam: look for interstitial keratitis
– Dilated fundus exam: search for chorioretinitis,
papilitis, and uveitis
– Lab tests: FTA-ABS or MHA-TP, RPR or VDRL
– Lumbar puncture
• Treatment
– Decision is based on whether active disease is
present and pt has been treated appropriately in
the past.
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Anisocoria

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41
 Neuro-ophthalmologic Disorders
• Clinical evaluation of NO patients
• Pupillary abnormalities
• Visual field defects
• Optic neuritis
• Papilloedema
• Optic nerve atrophy
• Disorders of cranial nerves
• Nystagmus
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VISUAL FIELD
DEFECTS

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 The Normal visual field
is defined as “Island of
vision surrounded by a
sea of blindness”
 The three dimensional
concept can be reduced to
quantitative values by
plotting lines (isopters) at
various levels around the
island or by measuring
the height (sensitivity) at
different points within the
island of vision.

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THE NORMAL EXTENT OF FIELD
OF VISION
 50°superiorly
 60°nasally.
 70°inferiorly .
 90° temporally

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COMMON CAUSES OF VF DEFECT
 Central field loss occurs with:
 Optic neuropathy
 Macular degeneration
 Macular hole
 Cone dystrophies
 A number of rare conditions like Best’s disease, Stargardt's disease and
achromatopsia.
Normal central ceco-central

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Peripheral field loss occurs with:
 Retinitispigmentosa
 Chorioretinitis
 Glaucoma
 Retinal detachment
 Leber's optic atrophy

Ring scotoma advanced glaucoma

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ASSESSING FOR VISUAL FIELD
DEFECTS CAN BE VIA
 Screening tests …
 confrontational visual field testing
 Amsler grid (assesses the central 10° the
visual field ) .
 Quantitative measurements using manual or
automated perimetry.

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 Visual acuity tests the eye's greatest power of
resolution .
 visual field testing measures the peripheral
sensitivity.
Static perimetry
 the most commonly used assessment .
 An 'on/off' light signal is presented throughout
the patient's potential visual field and the
patient clicks every time they see the signal.
 can assess various amounts of the visual field
(10° to full field).
 sensitive tests but are difficult to perform
 Humphries and octopus machines are most
commonly used.

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 Kinetic perimetry
-This presents a moving stimulus from a non-seeing
area to a seeing area.
-The most commonly used kinetic test is Goldmann
perimetry.
-It is repeated at various
points around the clock
and a mark is made as
soon as the point is seen.
These points are then
joined by a line
(an isopter).

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TERMS
Visual field defect - a portion of visual field
missing.
Scotoma - this is a type of visual field defect. It is
a defect surrounded by normal visual field.
 Relative scotoma - an area where objects of low
luminance cannot be seen but larger or brighter
ones can.
 Absolute scotoma - nothing can be seen at all
within that area.

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HEMIANOPIA - binocular visual defect in each eye's
hemifield.

 Bitemporal hemianopia -
the two halves lost are on
the outside of each eye's
peripheral vision,
effectively creating a
central visual tunnel.
 Homonymous
hemianopia- the two
halves lost are on the
corresponding area of
visual field in both eyes,
i.e. either the left or the
right half of the visual
field.

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 Altitudinal hemianopia -
refers to the dividing line
between loss and sight
being horizontal rather
than vertical, with visual
loss either above or below
the line.

 Quadrantanopia - is an
incomplete hemianopia
referring to a quarter of
the schematic 'pie' of
visual field loss.

 Sectoral defect - is also

an incomplete hemianopia

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 lesions before the chiasm
-These will produce a field deficit in the ipsilateral
eye.
-Field defects from damage to the optic nerve tend to
be central, asymmetrical and unilateral.
-Lesions just before the chiasm can also produce a
small defect in the upper temporal field of the other
eye.
lesions at the chiasm
These classically produce a bitemporal hemianopia.
 If they spread up from below, for example, pituitary
tumors, the defect is worse in the upper field.
 If the tumor spreads down from above , e.g.
craniopharyngioma, the lesion is worse in the lower
quadrants

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 lesions after the chiasm
-These produce homonymous field defects.
-A lesion in the right optic tract produces left
visual field defect.
-Lesions in the main optic radiation cause
complete homonymous hemianopia without
macular sparing.
-Lesions in the temporal radiation cause congruous
upper quadrantic homonymous hemianopia
commonly with macular sparing.
-Lesions in the parietal radiation (rare) cause
inferior quadrantic homonymous hemianopia
without macular sparing.

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-Lesions in the anterior visual cortex (common)
produce a contralateral homonymous hemianopia
macular sparing .
-Lesions in the macular cortex produce congruous
homonymous macular defect
-Lesions of the intermediate visual cortex produce a
homonymous arc scotoma, with sparing of both
macula and periphery.

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58
 Occipital lobe lesions
-If both occipital lobes are injured then the
patient is in a state of cortical blindness.
-Some patients deny their blindness and attempt
to behave as if they have vision. This state of
denial of cortical blindness is called Anton's
syndrome.

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 Localising the lesion

• Monocular visual field defects indicate lesions

anterior to the optic chiasm
• Bitemporal defects are the hallmark of chiasmal
lesions
• Binocular homonymous hemianopia result from
lesions in the contra lateral post chiasmal region
• Binocular quadrantanopias reflect optic tract
lesions

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NEURO-OPHTHALMOLOGIC DISORDERS

 Clinical evaluation of NO patients

 Pupillary abnormalities
 Visual field defects
 Optic neuritis
 Papilloedema
 Optic nerve atrophy
 Disorders of cranial nerves
 Nystagmus

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 OPTIC NEURITIS
Optic neuritis is the term used for inflammation of
the optic nerve.
Symptoms
-Optic neuritis may be symptomatic or asymptomatic.
-Symptomatic cases present with a triad of symptoms:
loss of vision, ipsilateral eye pain, and dyschromatopsia.
-Loss of vision deteriorating over hrs(rarely) to days
(most commonly).Visual loss may be subtle or profound.
-Usually unilateral, but may be bilateral
-Age typically 18-45 yrs

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 . . . OPTIC NEURITIS
… Symptoms
-Orbital pain, especially with eye movement
-Acquired loss of color vision
-Reduced perception of light intensity
-Occasionally Uhthoff’s sign(visual deficit with exercise
or increase in body temperature)
-May have neurologic symptoms or an antecedent viral syndrome(e.g.
upper respiratory, GI)
Signs
-RAPD in unilateral or asymmetric cases
-Decreased color vision
-Central, cecocentral, arcuate or altitudinal VF defects
-Swollen disc with or without peripapillary flame-shaped
hemorrhages(papillitis-most commonly seen in children
& young adults) or a normal disc( retrobulbar optic
neuritis-more commonly in adults)
-Posterior vitreous cells may be seen
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Classification of optic neuritis
It can be classified both etiologically and
ophthalmoscopically
Etiological classification
I. Demyelinating - the most common cause.
2. Para infectious - following a viral infection or
immunization.
3. Infectious -may be sinus-related or associated
with cat-scratch fever,syphilis,Lyme disease,
cryptococcal meningitis in patients with AIDS and
herpes zoster.
4. Autoimmune -may be associated with systemic
autoimmune diseases.

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Ophthalmoscopic classification
1.Retrobulbar neuritis -the optic disc appears
normal. It is the most frequent type in adults and
is frequently associated with multiple sclerosis
(MS).
2. Papillitis – the optic nerve head is affected
primarily or secondary to contiguous retinal
inflammation. It is characterized by variable
hyperemia and edema of the optic disc and may
be associated with para-papillary flame-shaped
hemorrhages
3.Neuroretinitis- is characterized by papillitis in
association with inflammation of the retinal
nerve fiber layer.

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 Classification of optic neuritis
Retrobulbar neuritis Papillitis (hyperaemia Neuroretinitis (papillitis
(normal disc) and oedema) and macular star)

• Demyelination-most • Viral infections and • Cat-scratch fever

common immunization in children
(bilateral) • Lyme disease
• Sinus-related (ethmoiditis) • Demyelination (uncommon)
• Lyme disease • Syphilis • Syphilis

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. . . OPTIC NEURITIS
Work-up
1.History: determine pt’s age and rapidity of visual loss.
Previous episode? Pain with eye movement?
2.Complete ophthalmic and neurologic exam, including
pupillary assessment, color vision, evaluation for
vitreous cells, dilated retinal exam with optic nerve
assessment.
3.Check blood pressure
4.VF test
5.For atypical cases: CBC,ESR, RPR, MRI of brain and
orbit

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. . . OPTIC NEURITIS

Treatment
If pt seen acutely:
1. If vision 20/40 or better: Observation is indicated
2. If vision is 20/50 or worse: steroid

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NEURO-OPHTHALMOLOGIC DISORDERS

 Clinical evaluation of NO patients

 Pupillary abnormalities
 Visual field defects
 Optic neuritis
 Papilloedema
 Optic nerve atrophy
 Disorders of cranial nerves
 Nystagmus

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 PAPILLEDEMA
Papilloedema is swelling of the optic nerve head
secondary to raised intracranial pressure.
It is nearly always bilateral although it may be
asymmetrical.
All other causes of disc edema in the absence of
raised intracranial pressure are referred to as
disc swelling.

70
.
Causes of Raised
Intracranial Pressure
1.Space-occupying lesions
2. Blockage of ventricular
system
3. Obstruction of
CSF absorption
4. Benign intracranial
Hypertension
(pseudotumour cerebri)
5. Diffuse cerebral edema
6. Hyper secretion of CSF

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 . . . papilledema
Symptoms:
-Episodes of transient, often bilateral, visual loss (lasting
seconds) often precipitated by changes in posture
-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 papilledema
Signs:
-Bilaterally swollen, hyperemic discs ( in early papilledema,
disc swelling may be asymmetric) with blurring of the disc
margin, often obscuring the blood vessels.

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. . . papilledema
Signs
-Papillary or peripapillary retinal hemorrhages( often
flame-shaped)
-Loss of venous pulsations (20% of normal population do
not have)
-Dilated, tortuous retinal veins
-Normal pupillary response and color vision
-An enlarged physiologic blind spot
-As chronic papilledema progresses to optic atrophy,
hemorrhages and cotton wool spots resolve,
peripapillary gliosis and narrowing of peripapillary
retinal vessels occur. Loss of color vision, central VA ,
and peripheral VF also occur.

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 Early papilledema

• VA - normal
• Mild disc hyperemia
• Indistinct disc margins - initially nasal
• Mild venous engorgement
• Normal optic cup
• Spontaneous venous pulsation - absent
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Established (acute) papilledema

• 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 75
Longstanding (chronic) papilledema

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

• VA - severely decreased
• Mild disc elevation
• Indistinct disc margins
• Disc pallor with few crossing vessels
• Absent optic cup 77
. . . papilledema
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
4. LP
Treatment
- treat the underlying cause of elevated ICP

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 Optic disc edema
Is any optic nerve swelling in the absence of ↑ed ICP.
Ten clinical signs of optic disc edema can be seen by
direct ophthalmoscopy:
The 5 mechanical signs:
1.Elevation of the optic disc (3 D = 1 mm of elevation)
2.Blurring of the optic disc margins
3.Filling in of the physiologic cup
4.Edema of the peripapillary nerve fiber layer
5.Retinal or choroidal folds

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. . . Optic disc edema
The five vascular signs are:
1.Hyperemia of the optic disc (Kestenbaum's number
may be greater than 12)
2.Venous congestion (venous dilatation and tortuosity)
3.Peripapillary hemorrhages
4.Exudates in the disc or peripapillary area
5.Nerve fiber layer infarcts

80
NEURO-OPHTHALMOLOGIC DISORDERS

 Clinical evaluation of NO patients

 Pupillary abnormalities
 Visual field defects
 Optic neuritis
 Papilloedema
 Optic nerve atrophy
 Disorders of cranial nerves
 Nystagmus

81
 OPTIC ATROPHY
-Optic atrophy represents the permanent loss of retinal
ganglion cell axons in conjunction with retinal ganglion
cell death.
-Optic atrophy should not be considered a diagnosis; it is
a pathologic end-point that is clinically discernible but
does not imply cause.
-Optic atrophy may be due to injury of the optic nerve
head. However, because of anterograde and retrograde
degeneration, it may reflect upstream injury of the
retinal
ganglion cells or downstream injury of the posterior
optic nerve, optic chiasm, or optic tract.

82
. . . Optic atrophy
-Differentiating primary from secondary optic atrophy is
clinically useful.
PRIMARY OPTIC ATROPHY
-The essence of primary or simple optic atrophy is a loss
of optic nerve fibers with otherwise minimal disturbance
of the optic nerve head microanatomy.
-It occurs without antecedent swelling of the optic
nerve head
-Primary optic atrophy can be a consequence of any
injury of the retinal ganglion cell or its axon at the level
of the nerve fiber layer; the optic nerve head; the orbital,
intra canalicular, or intracranial optic nerve; the optic
chiasm; the optic tract; or the lateral geniculate nucleus.
83
Common causes of primary optic atrophy
-Following retro bulbar neuritis.
-Compressive lesions such as tumors and
aneurysms
-Hereditary optic neuropathies
-Toxic and nutritional optic neuropathies

84
. . . PRIMARY OPTIC ATROPHY
-All causes of primary optic atrophy result in a loss of
axons with some associated gliosis, a reduction in optic
nerve diameter, a diminution of number and size of blood
vessels, and a thickening of the connective tissue septa.
Fundus changes:
-Optic disc atrophy may vary widely in appearance from
slight temporal (disc) pallor to a chalk-white optic nerve
head
-in its severe form, is characterized funduscopically by a
pale optic disc with clearly delineated borders
-In some cases, the surface of the disc may appear waxy

85
. . . PRIMARY OPTIC ATROPHY
. . .Fundus changes
-The shaggy or gray, fuzzy gliotic reaction characteristic of
secondary optic atrophy is not seen overlying the disc or
its margins. The absence of this gliotic reaction defines
primary or simple optic atrophy and is the basis for its
clinical differentiation.
-The pink or rose color of the disc is absent in primary
optic atrophy because of decreased blood perfusion
-reduction in the number of ophthalmoscopically visible
small blood vessels crossing the disc margin
(Kestenbaum's
capillary number test( from normal 10 to 7 or less)
-constriction of the papillary or peripapillary blood vessels
may occur

86
SECONDARY OPTIC ATROPHY
-The distinction between primary and secondary optic
atrophy is important because the latter is usually a
consequence of severe disc edema .
-Secondary optic atrophy reflects the disorganized
appearance of the surface of the optic disc seen if axonal
injury occurs in association with severe edema or
inflammation at the optic nerve head.1
-
most commonly occurs in cases of severe papilledema, it
may also develop in cases of long-standing severe
orbital
inflammation

87
. . . SECONDARY OPTIC ATROPHY

Fundoscopic features:
-The optic disc is grayish instead of pink or
white, and the margins appear fuzzy and
blurred. The glial proliferation may be
sufficient to give a raised appearance to the
optic disc and its margins
-absence of the pink color of perfusion, a
reduction in Kestenbaum's number, and a
constriction of the vasculature.

88
89
NEURO-OPHTHALMOLOGIC DISORDERS

 Clinical evaluation of NO patients

 Pupillary abnormalities
 Visual field defects
 Optic neuritis
 Papilloedema
 Optic nerve atrophy
 Disorders of cranial nerves
 Nystagmus

90
3RD , 4TH & 6TH NERVE PALSY
Anatomy
 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 extra ocular
muscles.

91
... Anatomy of 3rd , 4th & 6th nerves
CN III  motor innervations 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  motor innervation for SO muscle
CN VI  motor innervation for LR muscle

92
93
 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.

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. . . 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

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. . . 3rd – nerve palsy
Etiology
A. Pupil-involved
-More common: aneurysm (particularly posterior
communicating artery)
-Less common: micro vascular disease( DM, HTN), tumor,
trauma, congenital
-Rare: uncal herniation, cavernous sinus mass lesion, orbital
disease, herpes zoster, leukemia
B. Pupil-spared
-more common: Micro vascular disease
-Less common: Cavernous sinus syndrome
C. Relative pupil-spared: Micro vascular disease
D. Aberrant regeneration present: Trauma, aneurysm,
tumor, congenital. Not micro vascular

96
. . . 3rd – nerve palsy
Work-up
-Complete ocular exam
-Full neurological exam
-Edrophonium chloride test when pupil is not
involved and myasthenia gravis is suspected
-CT scan/MRI of brain
-Cerebral angiography
-Immediate ESR if giant cell arteritis is suspected
Treatment
-Treat underlying abnormality
-If double vision, patch the involved eye
Patching not performed in children younger than 9-11
year( risk of ambylopia)

97
. . . 3rd – nerve palsy
Follow-up
-Pupil-involved 3rd nerve palsy: immediate
hospitalization and work-up
-Pupil-spared 3rd nerve palsy: if new, observe for
5-7 days for delayed pupil involvement, then
recheck every 6 weeks.
Regain function within 3 months.
If palsy does not reverse by this time or if an
additional neurologic abnormality develops, do MRI.

98
 4TH –NERVE PALSY
Symptoms
-Binocular vertical diplopia, difficulty reading,
sensation that objects appear tilted; may be asymptomatic
Signs
-Deficient inferior movement of an eye when attempting
to look and in.
-The involved eye is higher(hypertropic) when pt looks
straight ahead.
-The hyperopia increases when looking in the direction
of
uninvolved eye or tilting the head toward the ipsilateral
Shoulder
-The pt often maintains a head tilt toward the
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. . . 4th –nerve palsy
Etiology
-More common: trauma, vascular infarct(DM, HTN),
congenital, idiopathic, or demyelinating diseases
-Rare: Tumor, hydrocephalus, giant cell arteritis,
aneurysm
Treatment
-Treat the underlying disorder
-Patch one eye if symptomatic diplopia
-Prisms in spectacles may be prescribed for small chronic
hyper deviations
-Surgery
Bothersome double vision in primary or reading position, for
cosmetic purposes, or for head tilt.
100
 6TH –NERVE PALSY
Symptoms
-Binocular horizontal diplopia, worse for distance
than near, most pronounced in the direction of
paretic lateral rectus muscle.
Signs
-One eye does not turn outward(temporally)
-No restriction on forced-duction testing
-No significant proptosis

101
. . . 6th –nerve palsy
Etiology
ADULTS
-More common: vasculopathic( diabetes, HTN,
atherosclerosis), traumatic, idiopathic
-Less common: Increase intracranial pressure, cavernous
sinus mass, multiple sclerosis, sarcoidosis/vasculitis
CHILDREN
-Benign post viral
-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

102
. . . 6th –nerve palsy

Treatment
-Any underlying problem revealed by the
work-up; otherwise, pts are managed by
observation
-Patching the paretic eye

103
CAVERNOUS SINUS/SUPERIOR ORBITAL FISSURE SYNDROME
(MULTIPLE OCULAR MOTOR NERVE PALSIES)

-A special situation exists when more than one ocular motor

nerve is involved either unilaterally or bilaterally.
-Common locations for multiple infra nuclear nerve
involvement are the subarachnoid space and the cavernous
sinus–superior orbital fissure.
Symptoms:
-Double vision,
-eyelid droop,
-facial pain or numbness

104
. . . cavernous sinus/superior orbital fissure syndrome
Signs:
-Limitation of eye movement corresponding to any
combination of a 3rd , 4th , or 6th nerve palsy on one
side
-Facial pain and/or numbness corresponding to one or
more branches of 5th CN
-A droopy eyelid and a small pupil (Horner’s
syndrome); the pupil may be dilated if the 3rd CN is
involved.
-Proptosis may be present when the superior orbital
fissure is involved.

105
. . . cavernous sinus/superior orbital fissure syndrome
Etiology
-Arterio venous fistula(carotid-cavernous or Dural-cavernous)
-Cavernous sinus thrombosis
-Metastatic tumors to cavernous sinus
-Intra cavernous aneurysm
-Tolosa-Hunt syndrome( Acute inflammation of superior orbital
fissure or anterior cavernous sinus)
Treatment
-Depends on the cause
-Referral

106
NEURO-OPHTHALMOLOGIC DISORDERS

 Clinical evaluation of NO patients

 Pupillary abnormalities
 Visual field defects
 Optic neuritis
 Papilloedema
 Optic nerve atrophy
 Disorders of cranial nerves
 Nystagmus

107
 NYSTAGMUS
-Nystagmus is a repetitive, involuntary to-and-
fro oscillation of the eyes.
Generally occur as result of
1. Inability to Maintain Fixation,
2. Loss of inhibitory input on eye movement
control system
3. Loss of symmetric input from vestibular
pathways to ocular motor nuclei

108
It may be physiological or pathological.
Nystagmus that occurs in response to rotation of
an optokinetic drum or of the body in space is
normal and acts to preserve clear vision, thus
physiologic.
In pathological nystagmus, each cycle of
movement is usually initiated by an involuntary
defoveating drift of the eye away from the object
of interest followed by a returning refixational
saccadic movement.

109
Symptoms
-Asymptomatic unless acquired after 8 years of age, at
which point the environment may be noted to oscillate
horizontally, vertically, 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.
110
 Evaluation of Patients with Nystagmus
Patients have sensation of environmental movement, also called
Oscillopsia.
Nystagmus that occur in primary position degrades VA
Nystagmus patients should be asked for Hx of associated neurologic symptoms &
any family History of Abnormal eye movements.
Most of Nystagmus can be detected by careful attention to characteristics of
Oscillations, while Patient Fixates on a stationary target at distance & near

The Following questions should be addressed:

l. Is drift away from target slow mov’t - Nystagmus or Fast -Saccadic Intrusion
2. Do slow mov’t occur in one direction & fast mov’t in opposite direction – Jerk
If opposing mov’ts of equal speed - Pendular Nystagmus
3. what is direction of instability; Horizontal,Vertical or Torsional
4. what is effect of blocking Fixation? If It increase Nystagmus
Intensity – Vestibular. N or decrease Intensity- Congenital .N ?

111
. . . Nystagmus
Nystagmus may be congenital or acquired

A. Congenital
1.Infantile nystagmus
2.Latent nystagmus
3.Nystagmus Blockage syndrome

112
 1. Infantile nystagmus
-Onset at age 2-3 months , with wide swinging eye
Movements
-At age 4-6 months, small pendular eye movements are
added, and at age 6-12 months, jerk nystagmus and a
null point(a position of gaze where nystagmus is
minimized) develop.
-Compensatory head nodding develops at any point up to
age 20 yrs.
-Infantile nystagmus is usually horizontal and typically
dampens with convergence

113
. . . Infantile nystagmus
Etiology
-Idiopathic
-Albinism
-Aniridia
-Leber’s congenital 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

114
 2.Latent Nystagmus
-Latent nystagmus: occurs only when one eye is
viewing. Fast phase of nystagmus beats toward viewing
eye.
-Manifest latent nystagmus: occurs in children with
strabismus or decreased vision in one eye.
Non fixating or poorly seeing eye acts as an occluded
eye.

Treatment:
-Maximize vision by refraction
-Treat amblyopia if indicated
-Consider muscle surgery if symptomatic strabismus
exists.
115
 3. Nystagmus Blockage Syndrome
-Any nystagmus that decreases when fixating eye is in
adduction and demonstrates an esotropia to dampen
the
nystagmus.
Treatment:
- For large face turn, consider muscle surgery

116
B. 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.

117