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Caroline Tilikete, MD, PhD

Neuro-ophthalmology Unit
Hospices Civils de Lyon, University Lyon I
Lyon, France
caroline.tilikete@chu-lyon.fr

DIAGNOSIS AND TREATMENT OF


NYSTAGMUS
Disclosure

None related to the teaching course


Learning objectives

Understand the main stabilizing oculomotor


systems
Recognize nystagmus versus saccadic
intrusions/oscillations
Recognize the main mechanisms of
nystagmus
Know the possible pharmacological
treatments of nystagmus
Key message

Abnormal eye movements generally correspond to


nystagmus and saccadic intrusions or oscillations.
Oscillopsia is lacking when ocular instability
develops in a context of blindness or infantile
nystagmus.
Clinical assessment of abnormal eye movements
depends on the clinical context, particularly on
whether patients present overt symptoms or not.
A precise clinical description of the abnormal eye
movement can be helpful to identify the localization
of the lesions, or suggest a specific etiology and
pharmacological treatment.
Clinical case 1

A 15 yo patient comes to your consultation for


diagnosis of abnormal eye movement.
She has normal motor and cognitive
development.
The abnormal eye movement was discovered on
examination, following minor head trauma. The
patient does not complain of any oscillopsia.
The mother said she had seen this abnormal eye
movement since infancy.
Clinical case 1
Reasoning

1. What are the arguments for a nystagmus?


2. What are the main arguments for an infantile
(congenital) nystagmus?
STABILIZING OCULOMOTOR SYSTEMS

Maintain fixation in primary position of gaze:


fixation system
Maintain fixation in lateral gaze: neuronal
integrator
Maintain fixation while moving: vestibular
ocular reflex
1. Fixation system
Oculomotor noise
Slow phases
Tremor
Microsaccades
1. Fixation system
OBJECTIVE
Attenuate oculomotor noise
INVOLVES
Visual feedback and motor cerebellar control for tremor
and slow phases
Fronto-striatal and cerebellar network for saccadic
inhibition
DEFICIT MAY EXPLAIN
Pendular Nystagmus
Saccadic intrusions and oscillations

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2. NEURAL INTEGRATOR

Premotor
neurons Oculomotor
nuclei

Neural integrator
2. NEURAL INTEGRATOR
OBJECTIVE
Maintain ocular stability in eccentric gaze
INVOLVES
Motor network in brainstem and cerebellum
DEFICIT MAY EXPLAIN
Gaze evoked nystagmus
Pendular nystagmus
3. VESTIBULO OCULAR REFLEX
T O

Vestibular Oculomotor
nuclei nuclei

T
Head position
in space
Eye position in space

Time
Eye position in
orbit
O
3. VESTIBULO OCULAR REFLEX
OBJECTIVE
Maintain ocular stability during head movements
INVOLVES
Vestibular system
Cerebellar control
DEFICIT MAY EXPLAIN
Pathological peripheral and central vestibular
nystagmus
Vestibulo-ocular areflexia

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Clinical case 2

A 63 yo man, with history of hypertension,


dyslipidemia and smoking, comes in
emergency room for an acute vestibular
syndrome.
He is complaining of vertigo, ataxia and
nausea for 4 hours.
Clinical case 2
Clinical case 2
Clinical case 2
Reasoning

1. What are the main arguments for a


vestibular nystagmus?
2. What would be the arguments for a central
versus a peripheral vestibular nystagmus?
Clinical case 3

A 60 yo patient comes to your consultation


for progressive occurrence of ataxia and
vertical oscillopsia, starting 3 years ago.
No personal or familial history
On examination
Mild non specific ataxia
Nystagmus
Clinical case 3
Reasoning

1. What type of nystagmus is it?


2. Which topography do you suggest?
3. Is the nystagmus related to ataxia?
4. What kind of treatment could you propose?
Clinical case 4

A 65 yo man, with history of chronic alcohol


intoxication, came in the emergency room for
subacute ataxia, cognitive deficit and
oscillopsia
On examination you find:
Cerebellar ataxia with dysarthria
Confusional state
Nystagmus
Clinical case 4
Reasoning

1. What type of nystagmus is it?


2. Which underlying mechanism do you
suggest?
3. What are the other main etiologies of this
nystagmus?
4. What kind of treatment could you propose?
Clinical case 5

A 40 yo man, with history of secondary


progressive multiple sclerosis, comes to your
consultation for visual troubles. He had
resolving episodes of optic neuritis years ago
on both eyes. He has ataxo-spasmodic
syndrome going on for a few years.
Hes complaining of blurred vision.
Clinical case 5
Reasoning

1. What type of nystagmus is it?


2. Which underlying mechanism do you
suggest?
3. What are the other main etiologies of this
nystagmus?
4. What kind of treatment could you propose?
Clinical case 6

A 65 y.o. patient was admitted in hospital for


ataxia and oscillopsia, ongoing for 2 weeks
He had no personal history
Examination showed
Important ataxia
Segmental myoclonia
Abnormal eye movements
Clinical case 6
Reasoning

1. What type of abnormal eye movement is it?


2. Which underlying mechanism do you
suggest?
3. What are the main etiologies of this
abnormal eye movement?
References
Averbuch-Heller L, Tusa RJ, Fuhry L, et al. A double-blind controlled study of gabapentin and
baclofen as treatment for acquired nystagmus. Ann Neurol 1997;41:818-825
Leigh RJ. Clinical features and pathogenesis of acquired forms of nystagmus. Baillieres Clin Neurol
1992;1:393-416
Leigh RJ, Zee DS. The Neurology of Eye Movement. Fifth edition ed. Gilman S, Herdman WJ,
editors. Oxford: Oxford University Press; 2015
McLean RJ, Gottlob I. The pharmacological treatment of nystagmus: a review. Expert Opin
Pharmacother 2009;10:1805-1816
Pierrot-Deseilligny C, Milea D. Vertical nystagmus: clinical facts and hypotheses. Brain : a journal
of neurology. 2005;128(Pt 6):1237-46.
Straube A, Leigh RJ, Bronstein A, Heide W, Riordan-Eva P, Tijssen CC, et al. EFNS task force--
therapy of nystagmus and oscillopsia. European journal of neurology. 2004;11(2):83-9
Strupp M, Thurtell MJ, Shaikh AG, Brandt T, Zee DS, Leigh RJ. Pharmacotherapy of vestibular and
ocular motor disorders, including nystagmus. J Neurol. 2011;258(7):1207-22.
Tilikete C, Jasse L, Pelisson D, Vukusic S, Durand-Dubief F, Urquizar C, et al. Acquired pendular
nystagmus in multiple sclerosis and oculopalatal tremor. Neurology. 2011;76(19):1650-7.
Thurtell MJ, Joshi AC, Leone AC, Tomsak RL, Kosmorsky GS, Stahl JS, et al. Crossover trial of
gabapentin and memantine as treatment for acquired nystagmus. Ann Neurol. 2010;67(5):676-80

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