Neuro-Ophthalmology Review
by
Peter Mark G. Chao, MD
May 5, 2011
Outline
Optic Neuritis
Cranial Nerve III palsy (Oculomotor)
Cranial Nerve IV palsy (Trochlear)
Cranial VI palsy (Abducens)
• Pain with eye movement; disc edema
Optic Neuritis mild
(< 45 age) • Recovery over months
Unilatera NA –AION • Disc edema with hemorrhage
l (> 40 age) • Altitudinal VF difect; poor recovery
Arteritic • Pale disc edema; profound visual loss
AION (> 60 common
Optic Disc
• Systemic symptoms common
age)
Edema
Significa • Unilateral disease in both eyes
nt VF • Chronic papilledema: pale
Bilateral
swelling with peripheral VF loss
loss
Minimal • Papilledema (tumor, etc):
abnormal MRI
VF loss • IIH : normal MRI
Optic Neuritis
Introduction
Inflammation of the optic nerve
occurs in young adults 15-45 years of age
Female : Male = 5 : 1
Etiology is viral (children)
Clinical Course
Rapid decrease in acuity the first 2-3
days
↓VA becomes stable for the next 7-10 days
Gradual improvement of vision
(returning to normal in 2-3 months)
Symptoms
Acute unilateral blurring of vision
Painful eye movement
periocular, retrobulbar, or simply globe tenderness
Dyschromatopsia, decreased brightness sense,
Pulfrich phenomenon altered depth perception
Uhthoff’s sign dimming of vision with elevation of
body temperature (exercise, sauna, fever)
Clinical Signs
↓ visual acuity, color vision and contrast sensitivity
Central or cecocentral scotoma is revealed
(+) RAPD
Two forms:
1. Papillitis (+) disc swelling
2. Retrobulbar neuritis (-) disc swelling
A neurologic exam completes the physical evaluation.
Diagnostics
MRI of the head and orbits
At least 3 periventricular white matter lesions or plaques
Developing clinically definite MS at 5 year follow-up
VER in multiple sclerosis
the increased in latency and decreased amplitude seen in
optic neuritis
laboratory work-ups that will document associated
systemic illness
FBS, CBC, ANA, ACE, VDRL, FTA-ABS, ESR
Optic Neuritis Treatment Trial
Sample size: 455 patients with optic neuritis
Treatment groups
1. No treatment (placebo tablets)
2. Moderate dose oral prednisone (1
mg/kg/day) for 14 days
3. High dose IV methylprednisolone (1000
mg/day) for 3 days, followed by oral
prednisone (1 mg/kg/day) for 11 days
(IV and oral) visual recovery the fastest , BUT, at 1
year follow up no significant difference among the
NO, NO TO ORAL
three groups
STEROIDS ALONE!!!
(IV and oral) and have 2 or more typical
demyelinating white matter lesion on MRI lower rate
BETTER
of developing MS within 2 TO
years ofGIVE
follow up.. But this
benefit is no longer measurable after the 2 year period
NOTHING!!!
(oral steroids alone) higher rate of recurrence of
optic neuritis
Subjective and objective improvement in
visual field should be evident in 3 – 5 weeks
after onset.
Failure to improve may still be consistent
with optic neuritis, but other items should be
considered
• Differentials: Papilledema, Ischemic Optic
Neuropathy, Papillophlebitis, Leber’s
Hereditary Optic Neuropathy
Cranial Nerve Palsies
Cranial nerve III palsy
Innervations
Presentation
Ptosis
A B
Etiology
• Depends on the age
• Congenital cases birth trauma or a neurologic syndrome
• Children infection, post-viral illness, trauma, or pontine
tumors
• Adults ischemia or microvascular problems
About 10-30% of adult cases undetermined
80% = pupil-sparing (microvascular or ischemic)
20% = pupil involving (compressive lesion)
Pupillomotor Fibers
Aneurysm at the posterior
communicating artery and internal carotid
artery = most common cause of non-traumatic
isolated CN 3 paresis with pupillary involvement
Pupillomotor fibers of CN 3
in the outer layer
Close to the nutrient blood supply
Symptoms
Binocular diplopia
Eye deviation
Headache
Droopy eyelid
Diagnostics
Head and orbital CT scan and MRI
MRA pupil is involved
Laboratory:
FBS, CBC, ESR, VDRL, FTA-ABS, and
ANA
Check blood pressure for HPN
Do tensilon test to rule out myasthenia
Treatment
• Etiology dependent
• Pupil –sparing lesions should be followed-
up closely for pupil involvement during the
1st week
• Occlusion of affected eye diplopia
• Treat HPN and DM
The prognosis depends on the etiology. It is
usually poor, except microvascular palsies,
which tend to resolve within 1-2 months
Treatment
Recommendation for MRI :
The pupil becomes involved in the initial 5-7
days after onset
No significant improvement in 3 months
Develops signs of aberrant regeneration
Other neurological findings develop
Aberrant Regeneration
Fiber of one structure being hooked up to
fibers that terminate in another structure
Trauma or compression (aneurysm or tumor)
NEVER occurs in ischemic causes
Lid gaze dyskinesis
IR to levator (Pseudo von-Graefe’s sign)
MR to levator (reverse Duane’s syndrome)
Aberrant Regeneration
Pupil gaze dyskinesis
MR fibers to
pupillary sphincter
muscles (Pseudo
Argyll Robertson
pupil)
IR fibers to pupillary
sphincter muscles
Cranial nerve 4 palsy
Etiology
• Most commonly caused = trauma (30-40%)
• Microvascular disease (20%)
• Congenital (with long-standing head tilt)
• Idiopathic (30%)
• Rarely by a tumor, hemorrhage or aneurysm
Signs and Symptoms
• Binocular vertical diplopia, may have tilted
vision, blurred vision
• Adopt a head tilt toward contralateral side,
chin down position
V-pattern ET and excyclotorsion of >10
degrees is seen in bilateral cases
(-) Forced ductions
Do Parks-Bielchowsky three-step test
Parks-Bielchowsky Test
Is a sequence of measurements of vertical
ocular misalignment that logically isolates a
weak vertically acting muscle
Assumes that only one vertically acting
muscle is paretic
RIGHT hypertropia Right – Left – Right
LEFT hypertropia Left – Right - Left
The vertical rectus muscles and the oblique muscles
control both the vertical position of the eyes as well
as the torsional position (keeping the eyes straight
when the head is tilted)
RSR RIO LIO LSR
RIR RSO LSO LIR
ELEVATIO DEPRESSION INTORSION EXTORSION
N
Right Gaze L IO L SO
R SR R IR
Left Gaze L SR L IR
R IO R SO
Right Head L SR R IR
Tilt L SO R IO
Left Head Tilt R SR L IR
R SO L IO
Step 1:
Determine which is hypertropic
using the cover-uncover test
in the primary gaze
RSR RIO LIO LSR
RIR RSO LSO LIR
Step 2:
Determine whether the hypertropia
is greater in
left or right gaze
RSR RIO LIO LSR
RIR RSO LSO LIR
Step 3:
Determine whether the hypertropia
is greater in
left or right head tilt
RSR RIO LIO LSR
RIR RSO LSO LIR
Cranial nerve vi palsy
Etiology
Etiology is age dependent
Children tumors (pontine glioma) or
post-viral
Adults trauma and microvascular
disease
Signs and Symptoms
Horizontal binocular diplopia (worse at
distance)
• Inward deviation of eye
• (-) forced ductions
• Other neurologic deficits are present if CN
VI palsy is not isolated.
Diagnostics and Managament
Head and orbital CT scan and/or MRI/MRA
Child
History of pain, head trauma, cancer
Signs of meningitis,
Associated with other neurologic abnormalities
No improvement of isolated microvascular cases after 3-6 months.
Isolated and microvascular cases in adults do not initially
require neuroimaging
Laboratory tests include FBS, CBC, ESR, VDRL, FTA-
ABS, ANA
Check blood pressure
Consider doing Tensilon test
Diagnostics and Managament
Treatment is etiology dependent
Occlusion of the affected eye
Muscle surgery in long-standing, stable cases
Aneurysms, tumors, and trauma may require
neurosurgery
Treat underlying medical condition
Prognosis
Etiology dependent; microvascular palsies tend to
resolve within 3 months.
Reference
Martin TJ, Corbett JJ. Neuro-ophthalmology: the requisites in ophthalmology. Mosby Inc.
2007; Missouri, USA
Kline LB, Bajandas FJ. Neuro-ophthalmology review manual, 5 th ed. SLACK Inc. 2001; NJ,
USA
Here comes the next reporter…
