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Neuro-ophthalmology for Medical Student

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

Wednesday, October 31, 2012

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

Wednesday, October 31, 2012

The scope of Neuro-ophthalmology

Oculomotor system

Disconjugate eyes: diplopia

• • • • •

conjugate eye movement Saccadic system Pursuit system Vergence system Counter rolling system: VOR, Ocular fixation system

• • •

Visual perception system

Eyelids Pupils

Wednesday, October 31, 2012

The scope of Neuro-ophthalmology

Oculomotor system

Disconjugate eyes: diplopia

• • • • •

conjugate eye movement Saccadic system Pursuit system Vergence system Counter rolling system: VOR, Ocular fixation system

• • •

Visual perception system Visual loss

Eyelids Pupils

Wednesday, October 31, 2012

The scope of Neuro-ophthalmology

Oculomotor system

Disconjugate eyes: diplopia

• • • • •

conjugate eye movement Saccadic system Pursuit system Vergence system Counter rolling system: VOR, Ocular fixation system

• • •

Visual perception system Visual loss

Eyelids Pupils

Ptosis

Wednesday, October 31, 2012

The scope of Neuro-ophthalmology

Oculomotor system

Disconjugate eyes: diplopia

• • • • •

conjugate eye movement Saccadic system Pursuit system Vergence system Counter rolling system: VOR, Ocular fixation system

• • •

Visual perception system Visual loss

Eyelids Pupils

Ptosis Anisocoria

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Oculomotor pathway
• Supranuclear(UMN)
• FEF: horizontal conjugate gaze • Diffuse frontal and occipital: vertical conjugate gaze

• Nuclear (LMN) • CN III, IV, VI • Internuclear
• PPRF, abducen interneuron, MLF (Horizontal gaze) • riMLF, INC, PC (Vertical gaze)

• Infranuclear(LMN)
• • • • Faciculus Cranial nerve NMJ muscle

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Frontal eye fields

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Right frontal lobe infarct

Frontal lobe lesion: no diplopia - Destructive FEF lesion:

• eyes deviate to the lesion
- Excitatory lesion:

• eyes deviate to contralateral to the lesion
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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

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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

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Key features
• • • • •
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Nuclear and fascicular lesion

• • •

Brain stem sign: long tract sign, other CN involvement

Nerve lesion Neighborhood 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

The action and nerve supply of the extraocular muscles is demonstrated.

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Nuclear and nerve lesion

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CN III

The oculomotor nerve (cranial nerve III)

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CN IV

The course of the trochlear nerve in the pons

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CN VI

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

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A woman with acute diplopia for 2 weeks

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A woman with acute diplopia for 2 weeks

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

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A woman with acute diplopia for 2 weeks

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

“Pure Right CN VI palsy”
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A 55 Y.O. with DM, HT presented with acute diplopia for 2 days

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

Left LR palsy
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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”
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Bilateral LR could be pseudo sixth nerve palsy from IICP

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Third nerve palsy

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Multiple oculomotor nerve involvement

• Cavernous sinus syndrome • Superior orbital fissure syndrome

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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

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Superior orbital fissure syndrome

CN 3, 4, 6, V1
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Superior orbital fissure 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

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

2 weeks

2 months

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

2 weeks

2 months

in a patient with polyneuropathy, all CN can be involved causing total ophthalmoplegia
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A patient with diplopia for 1 week with gait ataxia and areflexia

2 weeks

2 months

Dx. Miller Fisher syndrome
in a patient with polyneuropathy, all CN can be involved causing total ophthalmoplegia
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Interneuclear lesion
Interneuclear ophthalmoplegia (INO): MLF lesion Bilateral INO : Bilateral MLF lesion One and a half syndrome: PPRF lesion + MLF lesion

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Unilateral MLF lesion
• “ internuclear ophthalmoplegia “
• Ipsilat. MR weakness • Ipsilat. abducting saccade • Contralat. adducting nystagmus • Dissociated nystagmus

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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
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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

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A man with sudden diplopia

WIBINO
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One and a half syndrome
• Combined lesion : PPRF and MLF • “ One and a half syndrome “
– Ipsilateral horizontal gaze palsy – INO

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Infranuclear lesion ;
disease of ocular muscle disease of NMJ

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Total ophthalmopathy in CPEO patient

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TRIO with Bilateral ptosis (MG)

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

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

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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

Horner’s syndrome

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

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MG with enhancing ptosis

Ptosis due to NMJ lesion: sign of fatiguability
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Nystagmus

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Nystagmus
• • • •
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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 centers for ocular pursuit

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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
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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 slow phase has increasing velocity exponential: central in origin (usual form of congenital nystagmus)

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A woman with periodic vertigo occur when changing position

“vestibular nystagmus”
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case study: a boy with subacute dizziness

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Conclusion

Oculomotor system

• • • • •

conjugate eye movement Saccadic system Pursuit system Vergence system Counter rolling system: VOR, Ocular fixation system

• • •

Visual perception system

Eyelids Pupils

Wednesday, October 31, 2012

Conclusion

Oculomotor system

Disconjugate eyes: diplopia

• • • • •

conjugate eye movement Saccadic system Pursuit system Vergence system Counter rolling system: VOR, Ocular fixation system

• • •

Visual perception system

Eyelids Pupils

Wednesday, October 31, 2012

Conclusion

Oculomotor system

Disconjugate eyes: diplopia

• • • • •

conjugate eye movement Saccadic system Pursuit system Vergence system Counter rolling system: VOR, Ocular fixation system

• • •

Visual perception system Visual loss

Eyelids Pupils

Wednesday, October 31, 2012

Conclusion

Oculomotor system

Disconjugate eyes: diplopia

• • • • •

conjugate eye movement Saccadic system Pursuit system Vergence system Counter rolling system: VOR, Ocular fixation system

• • •

Visual perception system Visual loss

Eyelids Pupils

Ptosis

Wednesday, October 31, 2012

Conclusion

Oculomotor system

Disconjugate eyes: diplopia

• • • • •

conjugate eye movement Saccadic system Pursuit system Vergence system Counter rolling system: VOR, Ocular fixation system

• • •

Visual perception system Visual loss

Eyelids Pupils

Ptosis Anisocoria

Wednesday, October 31, 2012

Thank you for your kind attention
Wednesday, October 31, 2012

The Neurologist CMU

Wednesday, October 31, 2012