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. 2006 Mar;2(1):58-65.
doi: 10.3988/jcn.2006.2.1.58. Epub 2006 Mar 20.

Upbeat nystagmus: clinicoanatomical correlations in 15 patients

Ji Soo Kim  1 , Bora Yoon, Kwang-Dong Choi, Sun-Young Oh, Seong-Ho Park, Byung-Kun Kim
Affiliations

Upbeat nystagmus: clinicoanatomical correlations in 15 patients

Ji Soo Kim et al. J Clin Neurol. 2006 Mar.

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.

Keywords: Neural integrator; Upbeat nystagmus; Vestibulo-ocular reflex.

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Figures

Figure 1
Figure 1
Brain MRIs of patients with upbeat nystagmus reveal various lesions from the lower medulla to the thalamus.
Figure 2
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.
Figure 3
Figure 3
Positional modulation of upbeat nystagmus in patient 14. Upbeat nystagmus reverses direction to downbeat on straight-head hanging.
See this image and copyright information in PMC

References

    1. Leigh RJ, Zee DS. The neurology of eye movements. 3rd edn. New York: Oxford University Press; 1999.
    1. Kumar A, Patni AH, Charbel F. The Chiari I malformation and the neurotologist. Otol Neurotol. 2002;23:727–735. - PubMed
    1. Zingler VC, Strupp M, Jahn K, Glaser M, Herberger S, Kretzschmar HA, et al. Upbeat nystagmus as the initial clinical sign of Creutzfeldt-Jakob disease. Ann Neurol. 2005;57:607–608. - PubMed
    1. Jay WM, Marcus RW, Jay MS. Primary position upbeat nystagmus with organophosphate poisoning. J Pediatr Ophthalmol Strabismus. 1982;19:318–319. - PubMed
    1. Sibony PA, Evinger C, Manning KA. Tobacco-induced primary-position upbeat nystagmus. Ann Neurol. 1987;21:53–58. - PubMed