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Upbeat Nystagmus

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917-5<br />

<strong>Upbeat</strong> <strong>Nystagmus</strong>


Eye Movements<br />

<strong>Upbeat</strong> nystagmus in primary gaze<br />

Horizontal gaze evoked nystagmus left ><br />

right<br />

No nystagmus on downgaze<br />

Saccadic pursuit in all directions.<br />

Square wave jerks


Dysmetria<br />

Marked saccadic hypermetria<br />

Right gaze to center overshoot<br />

(hypermetria) taking the eyes almost<br />

fully to the left<br />

Left gaze to center (hypermetria) taking<br />

the eyes almost fully to the right<br />

Upgaze to center hypermetria<br />

Downgaze to center hypermetria


Clinical Features of <strong>Upbeat</strong><br />

<strong>Nystagmus</strong><br />

Present in primary gaze usually increases<br />

on upgaze<br />

Slow phases may have linear-, increasing-,<br />

or decreasing-velocity waveforms<br />

Poorly suppressed by visual fixation of a<br />

distant target


Clinical Features of <strong>Upbeat</strong><br />

<strong>Nystagmus</strong><br />

Convergence may increase, suppress or<br />

convert to downbeat nystagmus<br />

Associated with abnormal vertical<br />

vestibular and smooth-pursuit responses,<br />

and saccadic intrusions (square-wave<br />

jerks) that produce a bow-tie nystagmus


<strong>Upbeat</strong> <strong>Nystagmus</strong><br />

Localizes to the Caudal Medulla with the<br />

lesion affecting the perihypoglossal group<br />

of nuclei including:<br />

nucleus intercalatus<br />

nucleus of Roller<br />

nucleus of pararaphales


<strong>Upbeat</strong> <strong>Nystagmus</strong><br />

More rostral brainstem lesions may<br />

interrupt the ventral tegmental tract<br />

containing projections from the vestibular<br />

nuclei that receive inputs from the anterior<br />

semicircular canal<br />

Involve the brachuim conjunctivum in the<br />

rostral pons and medulla.<br />

or


Etiology of <strong>Upbeat</strong> <strong>Nystagmus</strong><br />

Infarction of medulla or cerebellum and<br />

superior cerebellar peduncle<br />

Wernicke’s encephalopathy<br />

Multiple sclerosis<br />

Tumors of the medulla, cerebellum or<br />

midbrain<br />

Cerebellar degeneration or anomalies


Etiology of <strong>Upbeat</strong> <strong>Nystagmus</strong><br />

Brainstem encephalitis<br />

Creutzfeldt-Jacob disease<br />

Bechet’s syndrome<br />

Meningitis<br />

Thalamic arteriovenous malformation<br />

Transient finding in infants


Clinical Features of Torsional<br />

<strong>Nystagmus</strong><br />

Torsional jerk nystagmus (minimal vertical<br />

or horizontal components) present with<br />

eye close to central position.<br />

Slow phases may have linear-, increasing-<br />

, or decreasing-velocity waveforms<br />

Poorly suppressed by visual fixation of a<br />

distant target<br />

Exacerbated by changes in head position<br />

or vigorous head shaking<br />

Leigh JR and Zee DS. The Neurology of Eye Movements 4 th Edition.<br />

Oxford University Press, New York 2006 with permission


Clinical Features of Torsional<br />

<strong>Nystagmus</strong><br />

May be suppressed by convergence<br />

Often occurs in association with ocular tilt<br />

reaction or unilateral internuclear<br />

ophthalmoplegia<br />

May be precipitated or modulated by<br />

vertical smooth pursuit movements.<br />

Leigh JR and Zee DS. The Neurology of Eye Movements 4 th Edition.<br />

Oxford University Press, New York 2006 with permission


Etiology of Torsional <strong>Nystagmus</strong><br />

Syringobulbia, with or without<br />

syringomyelia<br />

Arnold-Chiari malformation<br />

Brainstem stroke (e.g., Wallenberg’s<br />

syndrome)<br />

Arteriovenous malformation in the<br />

brainstem or middle cerebellar peduncle<br />

*Often occurs in association with the ocular tilt reaction and unilateral<br />

internuclear ophthalmoplegia.<br />

Leigh JR and Zee DS. The Neurology of Eye Movements 4 th Edition.<br />

Oxford University Press, New York 2006 with permission


Etiology of Torsional <strong>Nystagmus</strong><br />

Brainstem tumor<br />

Multiple sclerosis<br />

Oculopalatal tremor (“myoclonus”)<br />

Head trauma<br />

Congenital


References<br />

The Neurology of Eye Movements, 4 th Edition,<br />

Oxford University Press, New York, 2006.<br />

Tilikete C. Koene A. Nighoghossian N, Vighetto<br />

A, Pelisson. Saccadic lateropulsion in<br />

Wallenberg syndrome: a window to access<br />

cerebellar control of saccades? Exp Brain Res<br />

2006;174(3):555-565.


Tilikete C, Hermier M, Pelisson D, Vighetto<br />

A. Saccadic lateropulsion and upbeat<br />

nystagmus: disorders of caudal medulla.<br />

Ann Neurol. 2002 Nov;52(5):658-62.


http://library.med.utah.edu/NOVEL

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