Neuro Ophthalmology for Med Student_2016

7/21/2019 Neuro Ophthalmology for Med Student_2016

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Clinical Neuro-Ophthalmology

Surat Tanprawate, MD, MSc(London), FRCP(T)

Neurology Unit, Department of MedicineChiang Mai University

Slide download: FB: openneurons



The scope of

Neuro-Ophthalmology

• Oculomotor system

• conjugate eye movement

• Saccadic system

• Pursuit system

•Vergence system

• Counter rolling system:

VOR, Ocular fixation

system

• Visual perception system

• Eyelids

• Pupils



Oculomotor

pathway

• Supranuclear(UMN)• FEF: horizontal conjugate gaze

• Diffuse frontal and occipital:vertical conjugate gaze

• Nuclear (LMN)

• Nerve III, IV, VI Nucleus• Internuclear

• PPRF, abducen interneuron,MLF (Horizontal gaze)

• riMLF, INC, PC (Vertical gaze)

• Infranuclear(LMN)

• Fasciculus• Cranial nerve

• NMJ

• Muscle



Frontal eye fields



Frontal lobe lesion: no diplopia

- Destructive to FEF lesion:

• eyes deviate to the lesion- Destructive to Pontine lesion:

• eyes deviate contralateral to the lesion

- Excitatory lesion:

eyes deviate contralateral to the lesion

Right frontal lobe infarct



Case





Dysconjugate eyes



Diplopia (double vision)

• Diplopia is the simultaneousperception of the two images

of a single object that maybe displaced horizontally,vertically, diagonally

•caused by impair EOMs

functions

pic from wikipedia



Diplopia

Monocular

diplopia

Binocular

diplopia

Repetitive

images

Ghosting

image

- Cerebral polyopia

- Non-organic

- Retinal disease

- Refractive error

Misalignment of

the eyes

Nuclearcontrol

Internuclearcontrol

Infranuclearcontrol

- CN III

- CN IV

- CN VI

- CN palsy

- NMJ disorder

- Muscle disorder

Horizontal diplopia

- INO

- PPRF

Vertical diplopia

- INC, riMLF



Infranuclear control

Fasciculus

Nerve

NMJ

Muscle



emedicine.medscape.com/article/

IO SR IO/SR SR/IO SR IO

LR MR MR LR

SO IR SO/IR IR/SO IR SO

MR MR

CONVERGENCE



Ophthalmotrope (Ruete, 1857)

bjo.bmj.com/content/93/5.cover-expansion



IIIIV

VI

Nuclear and Internuclear control

Vertical gaze

internuclear control

Horizontal gaze

internuclear control

Nuclear control:

Nucleus III, IV, VI



Steps to exam patient with

diplopia1. Monocular vs Binocular diplopia

2. Exam eye movement: primary position and EOM

3. Other finding

a.eye lids

b.pupils

c.other cranial nerve

4. Specific findings/tests: fatigue test, weakness distribution,

reflex, typical facial features, etc



The action and nerve supply of the extraocularmuscles is demonstrated



Key featuresNuclear and fascicular lesion

• Brain stem sign: long tract sign, other CN involvement

Nerve lesion

• Neighbourhood sign; other CN, other sign

Internuclear lesion

• Specific syndrome; Internuclear Ophthalmoplegia (INO),

WEBINO, One and a half syndrome

NMJ lesion

•Fatiguability, not consistent with CN lesion, sign of

myasthenia gravis

Muscle lesion

• Not consistent with CN lesion: not consistent with CN lesion,sign of myopathy



Nuclear and nerve

lesion



The oculomotor nerve (cranial nerve III)

CN III



Feature of CN III palsy• Clinical features: Ophthalmoplegia(MR, SR, IR, IO),

Ptosis, Pupillary dilatation

• Part

• nuclear complex->fasciculus->basilar-

>intracavernous->intraorbital

• pupillomotor fibres



Stroke syndrome of CN IIIpalsy

1. Weber’s syndrome

2. Benedikt’s syndrome

3. Nothnagel’s syndrome

4. Claude’s syndrome



Weber’ssyndrome

• Contralateral

hemiparesis

• Ipsilateral CN IIIpalsy

• +/- contralateral

parkinsonism,

corticobulbar palsy

• cause: stroke, mass

lesion

http://www.cram.com



Benedikt’s syndrome(paramedian midbrain

syndrome)

• Ipsilateral CN III

palsy

• Tremor (red nucleus)

• Contralateral

extrapyramidal sign

http://www.cram.com

Note: Nothnagel: ipsi CN III palsy+cerebellar ataxiaClaude: Benedikt + Nothnagel



Isolated CN III palsy

Pupils sparing vs non-pupils sparing





Posterior communicating artery aneurysm

causing CN III palsy



Isolated CN III palsy with

sparing pupil in ischemic nerve



Neutral position

Upward

Downward

Right gaze Left gaze

Direct light reflex





Oculomotor nuclearcomplex lesion





Key finding of nuclear

complex CN III lesion

• incomplete involve muscle innervated with CN III

• +/- ptosis

• +/- pupillary involvement

• +/- other brain stem sign



The course of the trochlear nerve in the pons

CN IV



SO function• Depression

• Intorsion

• Abduction IOSR

SOIR



Head position in Forth nerve palsy

Head tilt chin down to unaffected side



Parks three steps to identifyCN IV palsy

1.which eye is higher in primary gaze?

2.Worse in right/left gaze?

3.Which head tilt gives greater hyperdeviation?

• “Left-Right-Left”

• Left SO palsy

• “Right-Left-Right”

• Right SO palsy



“Left SO palsy”



Cause of isolated CN IV in

adult

• 30% Unknown

• 20% Ischemic

• 10% Aneurysm

• 40% Traumatic

• CN IV is the longest and thinnest CN, and passes over the

tentorium cerebelli



facial nerve wraps around the nucleus of cranial nerve VI within

the pons

CN VI



• Part: nuclear->fasciculus->basilar(subarachnoidbase od skull, petrous bone)—>intracavernous->intraorbital



Stroke syndrome related toCN VI palsy

1. Foville syndrome

2. Millard-Gubler syndrome



Millard-Gubler syndrome (ventral pontine syndrome)

1. CN: ipsilat CN VI + CN VII

2. Corticospinal tr (contralat

hemiparesis)

“Cross hemiplegia”



Foville’s syndrome (inferior medial pontine syndrome)

1. CN VI, VII (ipsilat.)

2. Corticospinal tr. (hemiparesis)

3. Spinothalamic tr. (contralat.

hemisensory loss)

4. PPRF (lateral gaze weakness)



Basilar portion of CN VIpalsy

1. Acoustic neuroma: hearingloss+CN VI palsy (first sign isdiminished corneal sensitivity)

2. IICP3. Nasopharyngeal tumours:

invade the skull4. Basal skull fracture5. Gradenigo syndrome: acute

petrositis (CN VI + CN VIIpalsy + hearing loss + Pain)

!"#$%&'() )+",-+"# &. /+#0-%"$& '1%0+&2-Suppurative otitis mediaPain in the distribution of the trigeminal nerveAbducens nerve palsy



Bilateral LR could be pseudo sixth nervepalsy from IICP



Isolated CN VI palsy



Multiple nerve involvement

• Cavernous sinus syndrome

• Superior orbital fissure syndrome



29

Cavernous sinus syndrome

• Association with – other cranial nerve involvement:

4, 5, 6 CN

– oculosympathetic paralysis

– Opthalmic branch of trigeminal

nerve

• Tend to be partial; alls

muscles innervated are not

equally involved



30

Superior orbital fissure

syndrome

CN 3, 4, 6, V1



31

Superior orbital fissure syndrome

• Involve CN 3, 4, 6 and V1 CN 5distribution +/- oculosympatheticparesis without anhydrosis

• May exopthalmos due to

blockade of the opthalmic veins• Blindness due to extension of

the pathologic process toinvolve the optic canal





Interneuclear

lesion

Interneuclear ophthalmoplegia (INO): MLF lesionBilateral INO : Bilateral MLF lesionOne and a half syndrome: PPRF lesion + MLF lesion

Horizontal



Unilateral MLF lesion

• “ internuclearophthalmoplegia “• Ipsilateral MR weakness ipsilateral side

• Contralat. abducting nystagmus



Interneuclear ophthalmoplegia (INO)

c. Normal left abduction onleft gaze

d. Normal convergence

a. Normal primaryposition

b. Left impaired adductionon right gaze and horizontalnystagmus of the right eye



Bilateral MLF lesion

• Bilateral MLF lesion –Bilateral adducting weakness

–Bilateral abducting nystagmus –Impaired vertical vestibular and pursuit

–Impaired vertical gaze holding

–Gaze evoked nystagmus

• Wall eyed bilateral INO : WEBINO –exotropia



One and a half syndrome

• Combined lesion :PPRF and MLF

• “ One and a half

syndrome “ –Ipsilateral horizontal gaze

palsy

–INO





Bilateral PPRF lesion

• Bilateral horizontal gaze failure

• Sparing vertical gaze

• Sparing pupil

•May combine with other brain stem sign



Interneuclear lesion

Upward and downward gaze failure

Vertical



Vertical gaze control

A iddl ith t di i



A middle age woman with acute dizziness

Firstly, she was diagnosed as exophthalmos

and tested for TFT (but normal)



D l idb i d



Dorsal midbrain syndromeParinaud’s syndrome

• A group of eye abnormalities and pupillary dysfunction caused

by lesions of the dorsal midbrain

• Clinical syndrome

• Upward gaze palsy (supranuclear)

• Pseudo-Argyll Robertson pupils: light-near dissociation

• Convergence-Retraction nystagmus/ convergence spasm

• Eyelid retraction (Collier’s sign)

• “Setting sun” sign (conjugate down gaze in primary position)



cases

• compression

• ischemia/hemorrhage

• obstructive hydrocephalus

• infection

• tumour



Syndrome ofophthalmoparesis

• Miller-Fisher syndrome

• Wernicke encephalopathy



Wernicke’s encephalopathy

• Triad

• ophthalmoparesis/nystagmus

• acute confusion

• ataxia

A ti ti t ith NG t b f d



A pancreatic cancer patient with NG tube feed

for 3 months

She develop confusion with ataxia and dizziness







Victor M, et al. The Wernicke-Korsakoff Syndrome and Related Neurologic Disorders Due to

Alcoholism and Malnutrition. 2nd ed. 1989.



Treatment regime• Thiamine IV is recommended

• No consensus the dose and duration

• IV route

• Although standard recommended dose interval is oncedaily but half life is 96 mins so may need multiple timedaily

• Standard dose is 100 mg iv

• EFNS task force: 200 mg three times daily

R Galvin et al. European Journal of Neurology 2010, 17: 1408–1418



Infranuclear lesion ;

disease of NMJdisease of ocular muscle

Neuromuscular Junction



Neuromuscular Junction

Features of NMJ



Features of NMJdisorder

• Ophthalmoplegia is not consistent withnerve distribution

• Fatigue

• Fluctuating course

•with other muscle weakness esp. ptosis,

proximal muscle weakness



Eyelid and ptosis



• Upper eyelid –Levator palpebralsuperioris(CN 3)

–Mullermuscle(sympathetic)

–Frontalis muscle(CN 7)

• Lower eyelid –Capsulopalpebral

fascia(inferior rectus) –Inferior tarsal

muscle(sympathetic)

Ptosis



Ptosis

Neurologic ptosis

Non-neurogenic(mechanical)ptosis

•Uni-bilateral •Partial-complete

•Pupil involvement •EOM impairment

Supranuclearlesion(cerebralptosis) •Contralateralcerebral hemisphere

LMN •Neuropathic(N,fascicle, CN) •NMJ •Myopathic

Congenital ptosis

Horner’ssyndrome



Ptosis from Cranial nerve III lesion

- complete or near complete ptosis

- EOM involvement

- Pupil dilatation



Horner’s syndrome

• miosis

• ptosis (incomplete, upside down)

• anophthalmos

• anhidrosis









Afferent visual pathway

and visual loss









Visual loss

• Assessment

•

Visual acuity

• Pupillary reflex

• Visual field

• Fundus

Visual loss



Neurologic causes

Non-neurologicalcauses

-refractive error

-corneal problem -cataract

-glaucoma

-retinal and choroidaldisease

Diplopia

Type of visual fielddefect

Anterior visual pathway -Prechiasmatic lesion

-Chiasmatic lesion

-Retrochiasmatic lesion

Posterior visual pathway -LGB

-Geniculo-occipital lobepathway

-Occipital lobe

A t f i l l



Assessment causes of visual loss

• Unilateral or bilateral• Transient, non-

progressive, progressive• Sudden, gradual onset

• Sudden onset• Transient: monocular, binocular• Non-progressive: monocular,

binocular• Progressive : monocular, binocular

• Gradual onset

1) Visual loss of sudden onset



1) Visual loss of sudden onset

Unilateral transient visual loss• Ocular:

• angle closure glaucoma,hyphema, optic disc edema,partial retinal v. occlusion

• Retinal artery:

• vasospasm(migraine),• hypoperfusion(hypotension,

hyperviscosity,hypercoagulable stage),

• vasculitis(GCA),

• TIA(TMB, amaurosis fugax;emboli to retinal circulation)

• Disc: – trainsient visualobscuration(chronic swellingof optic disc)

• Optic nerve: – Uhthoff’s phenomenon in ON

Visual loss of sudden onset




Bilateral transient visual loss• Disc:

– papilledema(transient visual obscuration)

• Transient visual cortex dysfunction: – Decrease perfusion: thromboembolism, systemic

hypotension, hyperviscosity, vascularcompression

– Epilepsy

– Migraine





Non-progressive unilateral sudden visual loss• Hallmark of ischemic of optic nerve or retina

• CRAO, CRVO, AION

• Central serous choroidopathy• Retinal detachment

• Vitreous hemorrhage

• Functional visual loss





Non-progressive bilateral sudden visual loss• Occipital lobe infarct

• Pituitary apoplexy

• Functional visual loss• Head trauma

Visual onset of visual loss



Visual onset of visual loss

Sudden onset with progressive visual loss • Hallmark of inflammatory lesion: optic neuropathy • Ocular: low-tension glaucoma• Disc: papilledema• Anterior visual pathway:

– Inflammation: optic neuritis, – Hereditary: LHON – Toxic neutritional optic neuropathy – Compression: aneurysm, tumor, dysthyroid optic neuropathy – Radiation – Paraneoplastic retinopathy/optic neuropathy

Relative afferent pupillary reflex (RAPD)



(Marcus Gunn pupil) with swing flash light test



2) Visual loss of gradual onset



2) Visual loss of gradual onset

• Hallmark of compressive lesion • Affect: prechiasmal, chiasmatic visual pathway• Common: pituitary tumor, aneurysm, craniopharyngioma,

meningioma, glioma• Granulomatous involvement: TB, sarcoidosis• Ocular dysthyroidism

• Hereditary or degenerative of retina or optic nerve• Normal tension glaucoma• Chronic papilledema from pseudotumor cerebri• Medication: toxic to optic nerve• Radiation damage to anterior visual pathway

• Rapid pregressive paraneoplastic retinopathy/optic neuropathy



Pupillary and eyelidabnormality

Pupillary abnormality



Pupillary abnormality

• Evaluation of pupillary abnormality• Size and shape of pupil

• Reaction of pupil

– Light reaction• Direct light reflex

• Indirect(consensual) light reflex:swing flash light test

• Near(Accommodation) reflex:



100

Abnormal pupil



Abnormal pupil

• Size abnormality• Unequal size(anisocoria)

• Abnormal equal size:miosis VS mydriasis

• Shape abnormality

• Pupillary irregularity

• Abnormal pupillaryreflex

– Abnormal light reflex Abnormal direct light reflex

Abnormal consensual light

reflex: RAPD – Abnormal near reflex

– Light- near dissociation

Anisocoria



Neurologic anisocoria

Simple(physiologic) anisocoria

Opthalmologicanisocoria

Pupil dilatation pathwayabnormality

(anisocoria greater in

darkness) -Horner’s syndrome -old Adie’s(Tonic) pupil -Aberrent degeneration

Pupil constrictionpathway abnormality

-Adie’s pupil -CN3 palsy

Visualsystem

Symp: ptosis,anhydrosis

Parasymp: ptosis.EOM

Poorly reactive pupil without



anosocoria

• Large pupil – Hypothalmic lesion, midbrain lesion, syphilis,

botulism, MFS, autonomic neuropathy, drug/toxic,anxiety

• Small pupil – Old age, syphilis, diabetes, long standing Holme

Adie’s pupil, congenital, drug/toxic

Common pupil syndrome



Common pupil syndrome

• Adie Tonic pupil• Adie’s syndrome

• Light near dissociation

• Argyll-Robertson pupil

• Parinaud’s syndrome

• Horner’s syndrome

Tonic (Holme-Adie)pupil



Tonic (Holme-Adie)pupil

• Typically: unilateral mydriasis in healthyyoung women

• Acute: large

• Months to years: small

• React to light: sluggish or slow reaction tolight and slow(tonic) near response

• Cause: postganglionic parasympathetic

denervation

Light near dissociation



Light near dissociation

• Absent or impair light reflex with preservedaccommodation reflex and convergence

• Cause: – DM: small vv disease

– Dorsal midbrain syndrome

– Argyll-Robertson pupil

– Adie pupil

– Afferent visual pathway lesion

Argyll-Robertson pupil



Argyll-Robertson pupil

• Small, irregular, unequal• Normal afferent visual system

• Light near dissociation

• Cause: neurosyphilis



Pupillary irregularity



Pupillary irregularity

• Most: local disease of iris• Syphilis

• Ischemia

• Posterior synechiae

• Traumatic iridoplegia

• Degenerative disease of iris

• Holmes Adie syndrome

Localization of Horner’ssyndrome



syndrome



Nystagmus







Mechanism



Mechanism

•Nystagmus may result from dysfunction

of the vestibular ending organ,vestibular nerve, brainstem, cerebellum,or cerebral centre for ocular pursuit

Peripheral vs Central nystagmus



p y g

• Severe vertigo

• Minute to Day to weeks duration

• Hearing loss, tinnitus associated

• Usually horizontal with torsion

• Very rarely purely vertical or

torsional

• Commonly peripheral vestibular

organ dysfunction: labyrynthitis,

meniere’s disease

• None or mild vertigo

• Often chronic

• May be purely vertical or

torsional

• Visual fixation usually has no

effect

• Downbeat, upbeat, torsional

• Etiologies commonly vascular,

demyelination, pharmacologic,

toxic

Peripheral nystagmus Central nystagmus

A schematic illustration of nystagmus waveforms



In (A) a slow phase is followed by a slow phase while in (B)–(D)a slow phase is followed by a fast phase

(A) pendular nystagmus

(B) an accelerating velocityexponential slow phase jerknystagmus (CN)

(C) a decelerating exponentialslow phase jerk nystagmus(MLN)

(D) a linear or constant velocity

slow phase jerk nystagmus(MLN)

Mechanism



Mechanism

• Pendular nystagmus: is central (brainstem/ cerebellum)

• Jerk nystagmus:

• linear (constant velocity) slow phase: peripheralvestibular dysfunction

• slow phase has decreasing velocity exponential:brainstem neural integrator, cerebellar

• slow phase has increasing velocity exponential:central in origin (usual form of congenitalnystagmus)





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