NEURO-OPHTHALMOLOGY

OPEX REVIEW LECTURE

Eleonore B. Iguban, MD
May 27, 2010
OUTLINE
Optic Nerve Diseases
Anatomy
Signs of Optic Nerve Dysfunctiom
Optic Atrophy
Anterior Ischemic Optic Neuropathy
Papilledema
Leber Hereditary Optic Neuropathy
Optic Neuritis
 Anatomy of the Optic Nerve
Carries 1.2 M afferent
fibers originating from
the retinal ganglion cells

50 cm long from globe

to the chiasm
Anatomical Subdivisions of the Optic Nerve

Intra-ocular
Shortest (3-4mm)
Papillitis, optic disc
edema
Intra-orbital QuickTime™ and a
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Globe to optic foramen
Thicker-- + myelin
S-shaped
May cause pain on eye
movement when
inflamed
 Anatomical Subdivisions of Optic Nerve

Intracanalicular
Traverses the optic
canal
6mm
Fixed; dura mater
fuses with the
periosteum
Intracranial
Joins the chiasm
10mm
Signs of Optic Nerve Dysfunction
Reduced visual acuity
Afferent pupillary defect
Dyschromatopsia
Diminished light brightness sensitivity
Visual field defects
Primary Optic Atrophy
Caused by lesions from the
retrolaminar portion of ON to LGB
No antecedent swelling
Causes:
Compression of tumors/aneurysms
Retrobulbar neuritis
Hereditary, toxic, or nutritional optic
neuropathies
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Pale, flat disc
Distinct disc borders
 vessels on disc surface
(Kestenbaum sign)
Secondary Optic Atrophy
Preceded by swelling of
the optic nerve head
Causes:
Chronic papilledema
AION
papillitis
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White, dirty grey disc
Elevated disc
Indistinct disc borders
Non-Arteritic Anterior Ischemic Optic
Neuropathy (NAION)
Sudden, painless, monocular
Most common in visual loss not associated
elderly with premonitory visual
Infarction of optic obscurations
nerve head due to Predispositions:
HPN, DM
occlusion of short Sudden hypotensive episodes
posterior ciliary Sildenafil intake
arteries Collagen vascular diseases

6th-7th decade
Non-Arteritic Anterior Ischemic Optic
Neuropathy (NAION)
Signs:
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swelling with splinter-
shaped hemorrhages
Pallor ensues after 3-6
weeks
Fellow eye
involvement in 10%
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Arteritic Anterior Ischemic Optic
Neuropathy
Occlusion of the posterior
ciliary arteries due to
GCA
8th-9th decade
Sudden, profound visual QuickTime™ and a
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obscurations
Systemic signs of GCA
Arteritic Anterior Ischemic Optic
Neuropathy
Chalky white,
edematous optic disc
Optic atrophy ensues
in1-2 month
Tx:
Systemic steroids
 Papilledema
Passive, non-
inflammatory edema of
ONH
Secondary to increased
ICP
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No visual symptoms
Disc hyperemia, mild
elevation, slightly indistinct
borders
 Papilledema
Established
papilledema
Severe disc hyperemia
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hemorrhages and CWS
 Papilledema
Chronic Papilledema
“champagne cork” QuickTime™ and a
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Absent CWS,
hemorrhages
Atrophic papilledema
Greyish disc elevation
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 Papillitis vs Papilledema
Papillitis Papilledema
Unilateral Bilateral
Sudden onset Insidious onset
Sudden LOV Gradual LOV
Central scotoma Contraction of VF
Fine opacities seen in Clear posterior
posterior vitreous vitreous
Leber Hereditary Optic
Neuropathy
Mitochondrial DNA
mutation
2nd-4th decade, males
Unilateral, severe,
painless loss of central QuickTime™ and a
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Disc hyperemia, dilated
surface capillaries, and
swelling of peripapillary
nerve fiber layer
 Optic Neuritis
Inflammatory or demyelinating
Sudden loss of vision with ocular pain and
dyschromatopsia
Causes
Demyelinating disorders
Infectious
Systemic collagen vascular diseases
Intraocular Inflammation
 Optic Neuritis
Clinical Features: Fundus Examination
Retrobulbar Neuritis
Decreased visual acuity
ON normal
Decreased color vision Papillitis
Decreased contrast Swollen and hyperemic disc
Cells in posterior vitreous
sensitivity
Venous sheathing
+ RAPD Neuroretinitis
Central or arcuate field Disc edema
Macular star
defects
 Optic Neuritis

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 Optic Neuritis Treatment Trial
Patients treated with IV Methylprednisolone
followed by oral steroids recovered vision faster
Final visual outcome at 1 year is the same
Patients treated with oral steroids alone had a
higher rate of recurrences
After 15 years, 72 percent of patients with optic
neuritis had visual acuity of 20/20 or better
Optic Neuritis Treatment Trial

Methylprednisolone 1 g IV q6 for three

days followed by oral prednisone, 1 mg/kg
for 11 days
Dexamethasone 8mg IV LD then 4mg IV
q6 for three days followed by oral
prednisone, 1 mg/kg for 11 days
Post-Traumatic Optic Neuropathy
unexplained decrease in vision
With history of trauma
RAPD
Decrease color perception
Fundus:
Hyperemic disc
Indistinct, elevated disc borders
Tx: Steroids within 6 hrs of injury
 Oculomotor Nerve
Anatomy
Clinical Features
Lesions of the Oculomotor nerve
Nuclear Oculomotor Nerve Paresis
Oculomotor Nerve Fascicle Syndrome
Nothnagel’s
Benedict’s
Claude’s
Weber’s
Uncal Hernation Syndrome
PCOM Aneurysm
Cavernous Sinus Sydrome
Pupil-sparing Isolated Nerve Paresis
Cranial Nerve III

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Clinical Features
Profound ptosis
Abducted eye in
primary position
Limited adduction, QuickTime™ anddeco
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depression, elevation
Dilated pupils
Nuclear 3rd Nerve Paresis
Extremely rare
SR innervated by contralateral 3rd nerve
nucleus
Both levator palpebrae are innervated by
the central caudal nucleus = bilateral ptosis
 3rd Nerve Fascicle Syndrome

Nothnagel’s Claude’s
Superior cerebellar Nothnagel’s +
peduncle Benedict’s
cerebellar ataxia Weber’s
Benedict’s Pyramidal tract
Red nucleus Contralateral
Contralateral hemiparesis
hemitremor
 Uncal Herniation Syndrome
Caused by downward displacement and
herniation of the uncus--> CN 3
compressed
Hutchinson pupil- dilated and fixed pupil
seen in SOL
 PCOM Aneurysm

Travels along the PCOM in the cavernous

sinus
Pupil-involving CN 3 paresis
Pupil-sparing 3rd Nerve Paresis

Pupillomotor nerve bundles

outer layer
Rich blood supply
Seen in ischemic causes of CN 3 paresis
HPN
DM
Causes of CN 3 Palsy
Idiopathic -25%
Vascular
Aneurysm
Trauma
Tumors
Others ( syphilis, giant cell arteritis)
 Trochlear Nerve
Anatomy
Clinical Features
Special Test
Parks 3-step test
Bielschowsky’s Head Tilting test
Important Features
Only CN that emerges
dorsally
Crossed; innervates
contralateral SO QuickTime™ and a
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Very long and slender
Pathway of Trochlear Nerve

NUCLEUS
Midbrain at
inferior
colliculus QuickTime™ and a
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CAVERNOUS
SINUS
Below CN III,
above CN V1
Clinical Features
Hyperdeviation
Diplopia

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Clinical Features

Normal: eyes rotate in

opposite direction of tilt
･
Fourth nerve palsy QuickTime™ and a
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intentionally tilt
contralaterally so that
normal eye intorts and
lines up with affected
eye
 Parks-Bielchowsky 3-step testing
Step 1:
Hypertropic in primary position?
Step 2:
Hypertropia greater in R gaze or
L gaze?
WOOG (Worse on Opposite QuickTime™ and a
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Step 3:
Head tilt
BOOT (Better on Opposite Tilt)
 Abducens Nerve
Anatomy
Clinical Features
Lesions of the Abducens Nerve
Brainstem
Millard-Gubler Syndrome
Raymond’s Syndrome
Foville’s Sydrome
Subarachnoid Space
Petrous Apex Syndrome
Gradenigo’s Syndrome
Pseudo-Gradenigo’s Syndrome
Cavernous Sinus Syndrome
Orbital Syndrome
Course of Abducens Nerve
Clinical Features
Ocular deviation (esotropia)
Limited abduction
Diplopia
Head posturing
Head turned toward the side of the lesion

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Lesion in the Brainstem
Millard-Gubler Foville’s Syndrome
Syndrome
Passes through the
pyramidal tract (ventral)
Caused by vascular
disease, tumors,
demyelination
6th nerve paresis,
contralateral hemiplegia,
CN 7 nerve paresis
Passes near the PPRF,
MLF (dorsal)
involvement of CN V-
VIII, and central
sympathetic fibers
Facial analgesia,6th
nerve palsy, facial
weakness, deafness, and
central Horner’s
syndrome
 Lesion in the Subarachnoid and
Petrous Apex
Raised intracranial Gradenigo’s
pressure syndrome
Downward displacement Extradural abscess
of the brainstem following complicated
Stretches the CN VI otitis media
CPA tumors 6th nerve palsy,
decreased hearing,
Decreased hearing, facial pain, facial
decreased corneal paralysis
sensitivity, papilledema
 Lesion in the Intracavernous Sinus
Most medially located
Close to the ICA
Parkinson’s Sign
6th nerve palsy + post-ganglionic Horner’s
syndrome
Joined by the sympathetic branches of
paracarotid plexus
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Thank You!!!