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Vertical nystagmus in Wernicke’s encephalopathy: pathogenesis and role of central processing of information from the otoliths

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Abstract

Patients with Wernicke’s encephalopathy (WE) often have unusual patterns of vertical nystagmus. Initially there is often a spontaneous upbeating nystagmus that may change to downbeat nystagmus with a change in the direction of gaze, convergence or with vestibular stimuli. Patients also often show a profound loss of the horizontal but not the vertical vestibulo-ocular reflex (VOR). Furthermore, the acute upbeat nystagmus may change to a chronic downbeat nystagmus. We present hypotheses for these features based on (1) the location of vertical gaze-holding networks near the area postrema of the dorsomedial medulla where the blood–brain barrier is located, which we suggest becomes compromised in WE, (2) the location of the vestibular nuclei in the brainstem, medially for the horizontal VOR, and laterally for the vertical VOR, (3) neuronal circuits differ in susceptibility to and in the ability to recover from thiamine deficiency, and (4) impaired processing of otolith information in WE, normally used to modulate translational vestibulo-ocular reflexes, leads to some of the characteristics of the spontaneous vertical nystagmus including the peculiar reversal in its direction with a change in gaze or convergence.

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Author information

Correspondence to David S. Zee.

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Conflicts of interest

The authors declare that they have no competing interests.

Ethical approval

All experiments followed the tenets of the Declaration of Helsinki and this study was approved by the Institutional Review Board.

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The patient gave written consent to use this video for publication.

Additional information

This manuscript is part of a supplement sponsored by the German Federal Ministry of Education and Research within the funding initiative for integrated research and treatment centers.

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Baseline and Follow-up Video recording of case 3 (table 1). Baseline: The first video section was obtained three months after the diagnosis of WE, he has a robust UBN in straight-ahead gaze. In lateral gaze note DBN mixed with a horizontal component, right beat in right gaze and left beat in left gaze. Follow-up: The second Video section was obtained 10 weeks after the first recording: No nystagmus is present in straight-ahead gaze though with direct ophthalmoscopy and slit lamp examination there was a small-amplitude UBN. In lateral gaze he has a robust DBN (MP4 208212 kb)

Supplementary file1 (PDF 150 kb)

Baseline and Follow-up Video recording of case 3 (table 1). Baseline: The first video section was obtained three months after the diagnosis of WE, he has a robust UBN in straight-ahead gaze. In lateral gaze note DBN mixed with a horizontal component, right beat in right gaze and left beat in left gaze. Follow-up: The second Video section was obtained 10 weeks after the first recording: No nystagmus is present in straight-ahead gaze though with direct ophthalmoscopy and slit lamp examination there was a small-amplitude UBN. In lateral gaze he has a robust DBN (MP4 208212 kb)

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Kattah, J.C., McClelland, C. & Zee, D.S. Vertical nystagmus in Wernicke’s encephalopathy: pathogenesis and role of central processing of information from the otoliths. J Neurol 266, 139–145 (2019). https://doi.org/10.1007/s00415-019-09326-9

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Keywords

  • Thiamine deficiency
  • Vestibular
  • Nystagmus
  • Rotation
  • Translation