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Neuro-Ophthalmology

Celia H. Chang MD

Department of Neurology

MIND Institute

University of California, Davis, Health System

celia.chang@ucdmc.ucdavis.edu

Lecture Content

Disorders of optic nerve and retina

Chiasmal and retrochiasmal disorders

Pupil disorders

Motility disorders

2

Question #1

A 65-year-old woman has abrupt onset of visual loss in the right eye and weakness in the left arm and leg. There is some tingling of the left hand and left corner of her mouth. She is most likely to have a plaque in which blood vessel:

A. Anterior cerebral artery

B. Basilar artery

C. Internal carotid artery

D. Middle cerebral artery

E. Posterior cerebral artery

Question #2

A 35 year old woman woke with double vision. She reports that it gets better if she covers one eye. On exam: when looking to the left, her left eye has nystagmus to the left and her right eye does not move past the midline. She is able to look right without difficulty. She has normal vertical eye movements and is able to converge. The rest of her neurologic exam is normal. The most likely diagnosis is:

A. Cavernous sinus thrombosis

B. Midbrain lacune

C. Multiple sclerosis

D. Myasthenia gravis

E. Thyroid ophthalmopathy

Question #3

A 23 year old woman has the following visual field deficits.

Where is the lesion?

A. Optic radiation in the temporal lobe (Meyer's loop)

B. Optic nerve at the chiasm

C. Optic radiation in the parietal lobe

D. Lateral geniculate nucleus

E. Calcarine cortex of the occipital lobe

Question #4

A 60 year old man is unable to report seeing a comb and a fork at the same time even when the 2 objects are held next to each other. He also has poor coordination of hand and eye movements, and difficult tracking objects visually. What is the most likely diagnosis:

A. Holmes-Adie syndrome

B. Balint syndrome

C. Gerstmann syndrome

D. Parinaud’s syndrome

E. Malingering

Question #5

A 54 year old man has double vision. On exam, the left eye is deviated down and out. He also has ptosis and mydriasis. What is the most likely diagnosis:

A. Myasthenia gravis

B. Guillain-Barré syndrome

C. Posterior communicating artery aneurysm

D. Kearns-Sayre syndrome

E. Horner syndrome

Disorders of Optic Nerve and

Retina

Drusen

Optic neuritis

Papilledema

Retinal emboli

Retinal infarcts

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dralbertlin.com/resources/_wsb_326x326_OpticNerveNml01.jpg

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www.google.com/imgres?imgurl=http://webeye.ophth.uiowa.edu/eyeforum/atlas/photos/OND2.jpg&imgrefurl=http://webe

ye.ophth.uiowa.edu10

Optic Nerve Drusen

Refractile bodies

Frequently present at the nasal margin.

Visual field defects are common in patients

Afferent pupillary defect in asymmetric

cases

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www.eyes2health.com/images/optic%20neuritis.jpg

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www.uiowa.edu/~c064s01/nr122%20copy.jpg

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www.eyeweb.org/atlas/images/papilledema.jpg

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www.kellogg.umich.edu/theeyeshaveit/acquired/images/papilledema.jpg

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Pseudotumor Cerebri

Symptoms

Headache

Papilledema

Stabismus

Evaluate

Brain MRI with MRA and MRV

Lumbar puncture with opening pressure

Treatment16

en.wikipedia.org/wiki/File:Gray881.png17

http://www.insightseyecare.net/EyeEducation/retinaNeuronalLayers.jpg18

img.medscape.com/pi/emed/ckb/radiology/336139-417524-5003.jpg

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www.google.com/imgres?imgurl=http://www.kellogg.umich.edu

This refractile, yellow fleck is a platelet-

fibrin-cholesterol retinal embolus called a

Hollenhorst plaque20

uuhsc.utah.edu/moraneyecenter/opatharch/images/retina/22030.jpg

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Chiasmal and Retrochiasmal

Disorders

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en.wikipedia.org/wiki/Visual_system

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fc.units.it/ppb/neurobiol/Neuroscienze%20per%20tutti/vispath.gif

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www.jeffmann.net/NeuroGuidemaps/visualfields.gif

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www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cm&part=A3543

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Autonomic Control of Pupils

Target Sympathetic Parasympathetic

Pupil dilator

muscle

Alpha 1 contracts

• Mydriasis

M 3 relaxes

• Miosis

Ciliary muscle Beta 2 relaxes

• Long range

focus

M 3 contracts

• Short range

focus

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Pupil Disorders

Afferent pupillary defect (APD)

Horner

Tonic

Third nerve palsy

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Marcus-Gunn Pupil or Afferent

Pupillary Defect

Optic neuritis

Ischemic optic neuropathies

Glaucoma

Traumatic optic neuropathy

Radiation optic nerve damage

Miscellaneous optic neuropathies, such as

Leber's optic neuropathy

Ischemic retinal disease 29

Marcus-Gunn Pupil or Afferent

Pupillary Defect

Retinal detachmentif the macula or at

least two quadrants of retina

Severe macular degeneration

Not with cataract

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Argyll Robertson Pupil

Constrict when the patient focuses on a near

object (they “accommodate”)

Do NOT constrict when exposed to bright

light

Neurosyphilis

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www.ajnr.org/cgi/content/full/23/6/929/F1

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Horner’s Syndrome

Ptosis (superior tarsal muscle or Müller's muscle)

Miosis

Anhydrosis

Dilation lag

Enophthalmos

Iris hypopigmentation in congenital Horner’s due

to interference with melanin pigmentation

Lesion of cervical or thoracic sympathetic chain

Pancoast tumor (apical lung tumor)

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www.jeffmann.net/NeuroGuidemaps/Horners.gif

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Testing in Horner’s Syndrome

Cocaine test

Blocks the reuptake of norepinephrine

Normal pupil dilates

Horner’s: no dilation

Apraclonidine (alpha antagonist) reversal of

miosis on affected side

Paredrine test

Hydroxyamphetamine causes neurotransmitter

vesicle release if 3rd order neuron is intact

which results in mydriasis35

Holmes-Adie Syndrome or

Ross’s Syndrome

Mydriasis

Loss of deep tendon reflexes

Diaphoresis

Accommodative paresis

Photophobia

Ciliary and spinal ganglia

1/8% pilocarpine test

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img.medscape.com/pi/emed/ckb/ophthalmology/1189694-1198462-92.jpg

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Third Nerve Palsy

Down and out (infraducted and abducted)

Ptosis (levator palpebrae some bilateral

innervation)

Mydriasis (iris sphincter from ipsilateral

Edinger-Westphal subnucleus)

Posterior communicating artery aneurysm

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http://www.nature.com/eye/journal/v20/n10/images/6702376f10.jpg

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www.netterimages.com/images/vpv/000/000/006/6994-0550x0475.jpg

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Motility Disorders

Abnormal movement

Nystagmus

Other

Cranial nerve III, IV, VI paresis

Parinaud’s

Supranuclear gaze palsy

Internuclear ophthalmoplegia

One and a half syndrome

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Nystagmus Periodic rhythmic ocular oscillation of the

eyes

Equal amplitude and velocity (pendular

nystagmus)

Slow initiating phase and a fast corrective phase

(jerk nystagmus)

Horizontal nystagmus

Vertical nystagmus

Rotary

Congenital nystagmus

Acquired nystagmus42

Other Abnormal Eye Movements

Ocular bobbing

Ocular flutter

Opsoclonus

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img.medscape.com/pi/emed/ckb/clinical_procedures/79926-1412901-1189799-1585126.jpg

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www.pedseye.com/img/hyp_ex_02.jpg

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legacy.revoptom.com/handbook/images/62a.jpg

46

Paresis of Individual Ocular Muscles

Graves disease

Orbit pathology

Cavernous sinus pathology

Myasthenia gravis

Guillain-Barré syndrome

Mitochondrial disease

Myopathies

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webhard.gnu.ac.kr/WebLink/limbh/data/fig/no_parinaud.jpg

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Parinaud’s or Dorsal Midbrain

Syndrome

Paralysis of voluntary upgaze

Accommodative paresis

Convergenceretraction nystagmus

Eyelid retraction (Collier’s sign)

Conjugate down gaze in the primary

position

Pineal mass49

Progressive Supranuclear Palsy

Gait and balance problems

Difficulty with downgaze

Mood and behavior problems

Depression and apathy

Dementia

50

www.hmc.org.qa/qmj/qmj_nov_2006/images/Image_QMJ_2006/Fig12_63.jpg

51

http://www.google.com/imgres?imgurl=http://umed.med.utah.edu/

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Internuclear Ophthalmoplegia

Lack of conjugate adduction in the

ipsilateral MLF

Accommodation intact

Multiple sclerosis

Other pontine lesions such as CVA

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http://www.google.com/imgres?imgurl=http://umed.med.utah.edu

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Where pathway

What pathwayhttp://en.wikipedia.org/wiki/File:Ventral-dorsal_streams.svg

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Cortical Vision Disorders

Prosopagnosia

Inability to recognize familiar faces

Right occipitotemporallobe

Balint syndrome

Triad of:

Simultagnosia

Optic ataxia

Visual apraxia

Bilateral parietal lobe lesions

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References/Resources www.eyeweb.org

emedicine.medscape.com/ophthalmology

umed.med.utah.edu

Basics in neuro-ophthalmology by Kathleen B.

Digre, MD

Using the ophthalmoscope and viewing the

optic disc by Kathleen B. Digre, MD

www.jeffmann.net/Neuroguidemaps

uuhsc.utah.edu

www.kellogg.umich.edu

www.richmondeye.com57

Questions and Answers

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