Clinical Neuro-Ophthalmology

Surat Tanprawate, MD, MSc(London), FRCP(T)! Neurology Unit, Department of Medicine! Chiang Mai University

The scope of Neuro-Ophthalmology

Oculomotor system!

Disconjugate eyes: diplopia

conjugate eye movement! Saccadic system !

Visual perception system! Visual loss

!

Pursuit system! Vergence system! Counter rolling system: VOR, Ocular xation Anisocoria system

Eyelids! Pupils

Ptosis Anisocoria

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 elds

Right frontal lobe infarct

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

Case

Dysconjugate eyes

Diplopia (double vision)

Diplopia is the simultaneous perception of the two images of a single object that may be displaced horizontally, vertically, diagonally! caused by impair EOMs functions

pic from wikipedia

Diplopia
Monocular diplopia Binocular diplopia

Repetitive images

Ghosting image

Misalignment of the eyes

- Cerebral polyopia! - Non-organic

- Retinal disease! - Refractive error

Nuclear control

Infranuclear control

Internuclear control

- CN III! - CN IV! - CN VI

- CN palsy! - NMJ disorder! - Muscle disorder

Horizontal diplopia! - INO! - PPRF! Vertical diplopia! - INC, riMLF

Nuclear and Internuclear control

III

IV

VI

Nuclear control: Nucleus III, IV, VI Horizontal gaze internuclear control Vertical gaze internuclear control

Infranuclear control

Muscle Nerve NMJ

Fasciculus

Key features
Nuclear and fascicular lesion!

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

Nerve lesion! Neighbourhood sign; other CN, other sign!

Internuclear lesion! Specic 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

The action and nerve supply of the extraocular muscles is demonstrated

Nuclear and nerve lesion

CN III

The oculomotor nerve (cranial nerve III)

CN IV

The course of the trochlear nerve in the pons

CN VI

facial nerve wraps around the nucleus of cranial nerve VI within the pons

Isolated CN III palsy with sparing pupil

Cause of oculomotor nerve palsy

Common: vasculopathy (diabetes,
sinus thrombosis atherosclerosis, aneurysm), tumor!

Less common: inammation, cavernous

A woman with acute diplopia for 2 weeks

Right LR palsy; No other neurological sign, ! MRI brain-normal

Pure Right CN VI palsy

A 55 Y.O. with DM, HT presented with acute diplopia for 2 days

Left LR palsy
Dx. Left CN VI palsy from ischemic neuropathy

Bilateral LR could be pseudo sixth nerve palsy from IICP

Cause of CN VI palsy

Most common: vasculopathy (diabetes,

hypertension, atheroscleosis), trauma, idiopathic, IICP! cavernous sinus lesion, multiple sclerosis, vasculitis, stoke

Less common: giant cell arteritis,

Posterior communicating artery aneurysm causing CN III palsy

Multiple nerve involvement

Cavernous sinus syndrome! Superior orbital ssure syndrome

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

!29

Superior orbital ssure syndrome

CN 3, 4, 6, V1
!30

Superior orbital ssure syndrome

Involve CN 3, 4, 6 and V1 CN 5 distribution +/oculosympathetic paresis without anhydrosis! May exopthalmos due to blockade of the opthalmic veins! Blindness due to extension of the pathologic process to involve the optic canal

!31

A patient with diplopia for 1 week with gait ataxia and areexia

2 weeks

2 months

Dx. Miller Fisher syndrome

in a patient with polyneuropathy, all CN can be involved causing total ophthalmoplegia

Interneuclear lesion
Horizontal

Interneuclear ophthalmoplegia (INO): MLF lesion! Bilateral INO : Bilateral MLF lesion! One and a half syndrome: PPRF lesion + MLF lesion

Unilateral MLF lesion

internuclear ophthalmoplegia !
Ipsilateral MR weakness ipsilateral side! Contralat. abducting nystagmus

Interneuclear ophthalmoplegia (INO)

a. Normal primary position """""

b. Left impaired adduction on right gaze and horizontal nystagmus of the right eye

c. Normal left abduction on left gaze

d. Normal convergence

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

A man with sudden diplopia

WIBINO

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

A woman with diplopia and facial palsy

Interneuclear lesion
Vertical

Upward and downward gaze failure

Vertical gaze control

Cause of internuclear lesion

Common: demyelination (multiple
sclerosis), brainstem infarction!

Less common: tumor, infection

Infranuclear lesion ;
!

disease of NMJ ! disease of ocular muscle

Neuromuscular Junction

Features of NMJ disorder

Ophthalmoplegia is not consistent with
nerve distribution!

Fatigue! Fluctuating course! with other muscle weakness esp.

ptosis, proximal muscle weakness

A patient with diplopia and ptosis

Total ophthalmopathy in CPEO patient

TRIO with Bilateral ptosis (MG)

 Upper eyelid
Levator palpebral superioris(CN 3) Muller muscle(sympathetic) Frontalis muscle(CN 7)

Lower eyelid
Capsulopalpebral fascia(inferior rectus) Inferior tarsal muscle(sympathetic)

Ptosis
Non-neurogenic(mechanical) ptosis

Neurologic ptosis
Congenital ptosis

Uni-bilateral Partial-complete

Pupil involvement EOM impairment

! Supranuclear lesion(cerebral ptosis) Contralateral cerebral hemisphere

LMN Neuropathic(N, fascicle, CN) NMJ Myopathic

Horners syndrome

Ptosis from Cranial nerve III lesion! - complete or near complete ptosis! - EOM involvement! - Pupil dilatation

MG with enhancing ptosis

Ptosis due to NMJ lesion: sign of fatiguability

Nystagmus

Nystagmus

Ancient Greek (nustagmos (Ancient Greek,""nodding, be sleepy")! Involuntary biphasic rhythmic ocular oscillation in which one or both phase are slow! The slow phase is responsible for the initiation and generation of the nystagmus, whereas the fast (saccadic) phase i a corrective movement bringing the fovea back on target! Type: jerk (direction to fast phase) ; pendular nystagmus

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

Peripheral nystagmus

Central nystagmus

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, menieres disease

None or mild vertigo Often chronic May be purely vertical or torsional Visual xation usually has no effect Downbeat, upbeat, torsional Etiologies commonly vascular, demyelination, pharmacologic, toxic

A schematic illustration of nystagmus waveforms

(A) pendular nystagmus

(B) an accelerating velocity exponential slow phase jerk nystagmus (CN) (C) a decelerating exponential slow phase jerk nystagmus (MLN) (D) a linear or constant velocity slow phase jerk nystagmus (MLN)
In (A) a slow phase is followed by a slow phase while in (B)(D) a slow phase is followed by a fast phase

Mechanism

Pendular nystagmus: is central (brainstem/ cerebellum)! Jerk nystagmus: !

linear (constant velocity) slow phase: peripheral vestibular dysfunction! slow phase has decreasing velocity exponential: brainstem neural integrator, cerebellar! slow phase has increasing velocity exponential: central in origin (usual form of congenital nystagmus)

A woman with periodic vertigo occur when changing position

vestibular nystagmus

Case study: a boy with subacute dizziness

Conclusion

Oculomotor system!

Disconjugate eyes: diplopia

conjugate eye movement! Saccadic system !

Visual perception system! Visual loss

!

Pursuit system! Vergence system! Counter rolling system: VOR, Ocular xation system

Eyelids! Pupils

Ptosis Anisocoria

The Neurologist! CMU The Neurologist! CMU

Thank you for your kind attention