Introduction to

NeuroOphthalmology
Raed Behbehani , MD FRCSC

Neuroophthalmology
Diseases of the eye and the

neurological apparatus that serves it

(optic nerve and chiasm, cranial
nerves, visual pathways and cortex).

60% of our brain is linked to vision

Afferent: Optic nerve, retina, chiasm,
visual pathyways, cortx.

Efferent: Cranial nerve III,IV,VI, ocular

muscles, brain stem control centers.

Afferent System

Efferent System
Cranial
Nerves III, IV,
VI
Horizontal
and
Vertical
Gaze
Center
Smooth
Pursuit
and
Saccade

Symptoms
Loss of vision (transient, constant,
mono- or binocular).

Diplopia.
Ptosis.
Visual disturbances.
Pupil irregularities.
Eyelid or Facial spasms.

Clinical Approach
History is the most important part
or the assessment.

Where is the lesion ?

What can be the lesion ?
Is this an emergency ?

Diseases of the
Afferent System
Optic neuritis
Ischemic optic neuropathy (Arteritic vs
Non-Arteritic)

Other optic neuropathies (compressive,

papilledme, inflammatory, heriditary).

Chiasmopathies.
Strokes causing visual field defects.

Diseases of the
Efferent System
Cranial Neuropathies (III, IV, VI).
Nystagmus.
Ocular Myasthenia.
Blepharospasm, Hemifacial Spasm.
Pupillary Abnormalities.

What I can do for a

patient with vision
loss?
Before you refer, you can do a lot !
History : Sudden or chronic (urgency)
Check visual acuity (use near vision
cards).

Check for relative afferent pupillary

defect.

Do a visual field by confrontation.

Ophthalmoscopy.

How to check for

RAPD

Visual Field by
Confrontation

Direct
Ophthalmoscopy

Optic Neuritis
Sudden loss of vision.
Pain with eye movements.
Females > Males.
RAPD present.
Optic disc normal.
MRI is important for MS risk
determination.

MRI in optic
Neuritis

White matter lesion predicts high risk for

development of MS ( 70% over 15 years)

Ischemic Optic
Neuropathy

Age > 50.

Acute , painless, loss of vision.
Diabetes, hypertesnion, and
hyperlipedemia.

RAPD present.
Ophthalmoscopy : disc edema +hemorrhage.

Ischemic optic
neuropathy

Arteritic Ischemic
Optic Neuropathy

Patient > 60.

Headache, malaise, myalgia,

weight loss fever, jaw claudications,

and transient loss of vision.

ESR, CRP are high.

Need to start systemic steroids

immediately and do then do a TA

biopsy.

Temporal Arteritis

Retinal Artery
Occlusion

Painless loss of vision.

May be preceded by Amaurosis Fugax.
Source of emboli usually carotid or
cardiac.

Less common causes: Vasuclitis (GCA,

Anti-phospholipid syndrome).

Order Carotid Doppler Study and,

Echocardiography.

Central Retinal
Artery Occlusion

Branch Retinal
Artery Occlusion

Compressive
lesions

Slowly progressive loss of vision.

Can by uni-lateral or bilateral.
Pituitary tumors,
craniopahryngiomas, and
meningiomas of the skull base.

Require neuro-imaging (MRI) for

diagnosis.

Visual field defects

Pituitary tumors

Pituitary Tumors

Homonymous
Hemianopsia

Papilledema
Disc edema due to raised intracranial
pressure (mass, pseudotumor cerebri).

Headache, transient visual

obscurations, Diplopia, and tinnitus.

Normal visual acuity and visual fields

early.

Ophthalmoscopy.
Urgent CT scan of the head with
contrast.

Papilledema

Idiopathic Intracranial
Hypertension (pseudotumor
cerebri)

Women > Men (9:1) in childbearing age.

90% of affected women are obese.
Normal CT/MRI/MRV and CSF analysis.
Recent weight gain (last 6 months).
Medications-linked : Tetracycline for acne , oral
contraceptives, insulin-like growth factors in
children.

Aim of treatment is stop progressive loss of

vision (Diuretics and Surgery).

Diplopia
Key question Is it only in one

eye ? , Does it go away when you

close either eye ?

Monocular diplopia is always

refractive in origin (cataract,
astigmatism).

Examine lids and pupils in addition

to eye movement.

Examine all cranial nerves.

Oculomotor Nerve
Palsy

Pupil-involving
Third Nerve Palsy

UrgentMRI/MRA or MRI/CTA

Abducens Nerve
Palsy

Trochlear Neve
Palsy
Patients complain of vertical diplopia.
Can present with abnormal head tilt.
Can be congenital or acquired.

Trochlear Nerve
Palsy - Head Tilt
Test

Cranial
Neuropathies
(III,IV,VI)
Ischemic (diabetes, hypertension
and hyperlipidemia).

Demyelinating.
Compressive (tumor, aneurysm).
Trauma.
Raised ICP.

Multiple Cranial
Neuropathies
(III,IV,VI)

Ischemic cranial neuropathies are

almost always isolated.

If multiple simultaneous CN,

suspect lesion in the posterior

orbit/cavernous sinus region.

Usually due to mass lesion.

Cavernous Sinus

Ocular Myasthenia
Myasthenic signs restricted to the
ocular muscles.

Fatiguable diplopia and ptosis.

Ice test or rest test in the clinic
demonstrate improvement.

Acetylcholine receptor antibodies

(positive in 50 % only).

Single fiber EMG.

Ocular Myasthenia
before ice
test

after ice
test 2
minutes

Pupillary
Abnormalities

Anisocoria : Unequality of pupils size.

It can be accidental discovery.
Physiologic in 40% of patients
It can be isolated or associated with lid or
ocular motility abnormalities.

Can be iatrogenic or self-induced

(pharamacologic).

Pupil Examination
Shine light directly at pupil (light
response).

Test near response (miosis with

accomodation).

Check pupil sizes and measure it in

both light and dark.

Parasympathetic (constrict) and

Sympathetic (dilate) control.

Pupil Light Reflex

Diagnosis ?

Horner Syndrome
A defect in oculosympathetic flow to the
eye (pupil does not dilate in dark).

Ptosis, miosis and pseudo-enophtalmos.

Internal carotid artery dissection, neck
trauma or surgery, brain stem strokes
(Wallerburg Syndrome), Apical lung
tumors.

Urgent MRI/MRA of the head and neck

for acute Horners Syndrome.

Oculosympathetic
Pathway

Adies Pupil
Pupil is larger with light/near dissociation
(pupil does not constrict well to light but
does for near).

Can be associated with diminished deep

tendon reflexes (Holmes-Adies Syndrome).

Benign Essential
Blepharospasm

Hemifacial Spasm

Summary
Neuro-ophthalmic problems of the afferent
and efferent visual system are common.

Afferent diseases include optic nerve,

chisamopathies and visual pathway
diseases.

Efferent diseases include cranial

neuropathies, pupillary abnormalities and

facial spasms.

There is no substitute for good medical

history and examination.