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Generalized vestibular hyporeflexia and chronic upbeat nystagmus due to thiamine deficiency

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Abstract

Background

Ocular motor and vestibular manifestations of Wernicke’s thiamine deficiency (WTD) are frequent and heterogeneous. Previous neuropathological and neuroimaging findings identified brainstem and cerebellar lesions responsible for these findings, however, peripheral vestibular lesions are probably uncommon in human WTD, though noted on an avian thiamine deficient study.

Material

Single case study of a WTD patient post-gastric bypass who developed ataxia, oscillopsia and nystagmus, with low serum thiamine, and increased MRI T2 signal in the thalami, but normal brainstem and cerebellum. Vestibular evaluation showed significant vestibular hyporreflexia affecting all six canals, and a chronic upbeat nystagmus, now for 14 months after WTD onset.

Methods

Serial clinical, video head impulse, nystagmus analysis, cervical and ocular vestibular evoked responses. She is undergoing treatment with Memantine, Clonazepam and vestibular rehabilitation, and feels improvement.

Conclusion

This report shows a novel combination of central and peripheral vestibular findings, of relevance for diagnosis and treatment, in addition to the development of a coherent hypothesis on the ocular motor and vestibular findings in WTD.

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Data availability

Data from clinical, vestibular recording, eye movement recordings and video recording are avialable upon request.

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Author information

Authors and Affiliations

  1. Department of Neurology and Neurological Sciences, Neurotology, Stanford School of Medicine, Stanford, CA, USA

    William Zhu

  2. Department of Otolaryngology-Head and Neck Surgery and Department of Neurology and Neurological Sciences, Stanford School of Medicine, Stanford, CA, USA

    Kristen K. Steenerson

  3. Department of Neurology, University of Illinois College of Medicine, Peoria, IL, USA

    Jorge C. Kattah

Authors
  1. William Zhu
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  2. Kristen K. Steenerson
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  3. Jorge C. Kattah
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Corresponding author

Correspondence to Jorge C. Kattah.

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Zhu, W., Steenerson, K.K. & Kattah, J.C. Generalized vestibular hyporeflexia and chronic upbeat nystagmus due to thiamine deficiency. J Neurol 270, 1713–1720 (2023). https://doi.org/10.1007/s00415-022-11514-z

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  • DOI: https://doi.org/10.1007/s00415-022-11514-z

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