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Opsoclonus Following Downbeat Nystagmus in Absence of Visual Fixation in Multiple System Atrophy: Modulation and Mechanisms

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Abstract

We report atypical opsoclonus in a patient with multiple system atrophy and propose a mechanism based on the patterns of modulation by visual, vestibular, and saccadic and vergence stimulation. Firstly, the 6-Hz opsoclonus mostly in the vertical plane occurred only after the development of downbeat nystagmus in darkness without visual fixation. Even after a substantial build-up, visual suppression of the opsoclonus was immediate and complete. Furthermore, the latency for re-emergence of opsoclonus in darkness was greater when the duration of preceding visual fixation was longer. Secondly, the effect of preceding downbeat nystagmus on the development of opsoclonus was evaluated by changing the head position. The opsoclonus did not occur in the supine position when the downbeat nystagmus was absent. After horizontal head shaking, the opsoclonus in the vertical plane gradually evolved into horizontal plane and resumed its vertical direction again after vertical head shaking. Thirdly, any opsoclonus was not triggered by imaginary saccades in the supine position. Lastly, combined vergence and saccadic eye movements during the Müller paradigm did not induce opsoclonus. From these findings of modulation, we suggest that the opsoclonus observed in our patient was invoked by vestibular signals. When the function of the omnipause neurons and saccadic system was impaired, the centrally mediated vestibular eye velocity signals may activate the saccadic system to generate opsoclonus. These atypical patterns of opsoclonus, distinct from the classic opsoclonus frequently observed in para-neoplastic or para-infectious disorders, may be an unrevealing sign of degenerative brainstem or cerebellar disorders.

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Funding

This research was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Science and ICT (NRF-2017R1C1B1008582).

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Author notes

  1. Jeong-Yoon Choi and Ji-Soo Kim contributed equally to this study as the corresponding authors.

Authors and Affiliations

  1. Dizziness Center and Clinical Neuroscience Center, Seoul National University Bundang Hospital, Seongnam, South Korea

    Ju-Young Lee, Eunjin Kwon, Jeong-Yoon Choi & Ji-Soo Kim

  2. Department of Neurology, Seoul National University Bundang Hospital, 173-82 Gumi-ro, Bundang-gu, Seongnam, 13620, South Korea

    Ju-Young Lee, Eunjin Kwon, Jeong-Yoon Choi & Ji-Soo Kim

  3. Research Administration Team, Seoul National Univertisy Bundang Hospital, Seongnam, South Korea

    Hyo-Jung Kim

  4. Department of Neurology, College of Medicine, Seoul National University, Seoul, South Korea

    Jeong-Yoon Choi & Ji-Soo Kim

  5. The MTV (Migraine, Tinnitus, and Vertigo) Clinic, Oh Neurology Center, Daegu, South Korea

    Hui Jong Oh

Authors
  1. Ju-Young Lee
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Contributions

Dr. J.Y. Lee analyzed and interpreted the data and wrote the manuscript. Drs. H.J. Kim and H.J. Oh analyzed and interpreted the data and revised the manuscript. Dr. J.Y. Choi designed and conceptualized the study, interpreted the data, and revised the manuscript. Dr. J. S Kim interpreted the data and critically revised the manuscript.

Corresponding authors

Correspondence to Jeong-Yoon Choi or Ji-Soo Kim.

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Conflict of Interest

Dr. J.S. Kim serves as an associate editor of Frontiers in Neuro-otology and on the editorial boards of the Journal of Korean Society of Clinical Neurophysiology, the Journal of Clinical Neurology, Frontiers in Neuro-ophthalmology, the Journal of Neuro-ophthalmology, the Journal of Vestibular Research, and the Journal of Neurology, and Medicine. Others have no conflicts of interest to disclose.

Ethical Standard

This study followed the tenets of the Declaration of Helsinki and was performed according to the guidelines of the Institutional Review Board of Seoul National University Bundang Hospital (B-1109/135-106).

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Lee, JY., Kwon, E., Kim, HJ. et al. Opsoclonus Following Downbeat Nystagmus in Absence of Visual Fixation in Multiple System Atrophy: Modulation and Mechanisms. Cerebellum 20, 724–733 (2021). https://doi.org/10.1007/s12311-019-01090-w

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