J Clin Neurol.  2006 Mar;2(1):58-65. 10.3988/jcn.2006.2.1.58.

Upbeat Nystagmus: Clinicoanatomical Correlations in 15 Patients

• Kim JS
• Yoon B
• Choi KD
• Oh SY
• Park SH
• Kim BK

• KMID: 2287704
• DOI: http://doi.org/10.3988/jcn.2006.2.1.58

Abstract

BACKGROUND AND PURPOSE
The mechanism of upbeat nystagmus is unknown and clinicoanatomical correlative studies in series of patients with upbeat nystagmus are limited.
METHODS
Fifteen patients with upbeat nystagmus received full neuro-ophthalmological evaluation by the senior author. Nystagmus was observed using video Frenzel goggles and recorded with video-oculography. Brain lesions were documented with MRI.
RESULTS
Lesions responsible for nystagmus were found throughout the brainstem, mainly in the paramedian area: in the medulla (n=8), pons (n=3), pons and midbrain with or without cerebellar lesions (n=3), and midbrain and thalamus (n=1). Underlying diseases comprised cerebral infarction (n=10), multiple sclerosis (n=2), cerebral hemorrhage (n=1), Wernicke encephalopathy (n=1), and hydrocephalus (n=1). Upbeat nystagmus was mostly transient and showed occasional evolution during the acute phase. In one patient with a bilateral medial medullary infarction, the upbeat nystagmus changed into a hemiseesaw pattern with near complete resolution of the unilateral lesion. Gaze and positional changes usually affected both the intensity and direction of the nystagmus. A patient with a cervicomedullary lesion showed a reversal of upbeat into downbeat nystagmus by straight-head hanging and leftward head turning while in the supine position. Gaze-evoked nystagmus (n=7), ocular tilt reaction (n=7), and internuclear ophthalmoplegia (n=4) were also commonly associated with upbeat nystagmus.
CONCLUSIONS
In view of the responsible lesions and associated neuro-ophthalmological findings, upbeat nystagmus may be ascribed to damage to the pathways mediating the upward vestibulo-ocular reflex or the neural integrators involved in vertical gaze holding.

Keyword

Upbeat nystagmus; Vestibulo-ocular reflex; Neural integrator

MeSH Terms

Brain
Brain Stem
Cerebral Hemorrhage
Cerebral Infarction
Eye Protective Devices
Head
Humans
Hydrocephalus
Infarction
Magnetic Resonance Imaging
Mesencephalon
Multiple Sclerosis
Negotiating
Ocular Motility Disorders
Pons
Reflex, Vestibulo-Ocular
Supine Position
Thalamus
Wernicke Encephalopathy

Figure

• 

  Figure 1 Brain MRIs of patients with upbeat nystagmus reveal various lesions from the lower medulla to the thalamus.

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  Figure 2 Video-oculography in patient 14 reveals conjugate upbeat nystagmus with an initial counterclockwise torsional component (A), which becomes mainly a torsional component with near-complete resolution of the vertical component 5 months later (B). Upbeat nystagmus shows a decelerating slow phase.

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  Figure 3 Positional modulation of upbeat nystagmus in patient 14. Upbeat nystagmus reverses direction to downbeat on straight-head hanging.

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