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Downbeat nystagmus as an initial clinical sign in spinocerebellar ataxia type 6

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Correspondence to Tatsuya Ueno.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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In the sitting position, spontaneous downbeat nystagmus (DBN) was observed during primary gaze. The amplitude of DBN increased during lateral gaze, and decreased during downgaze. Smooth pursuit was slightly saccadic in all directions, although saccade was normal. The horizontal vestibulo-ocular reflex was impaired. Cancellation of the vestibulo-ocular reflex was also impaired. Using a charge coupled device camera, spontaneous DBN was also observed in the left eye in the supine position. Limb ataxia was not observed. The patient was able to perform the closed leg standing position with her open eyes. However, her standing position was slightly unstable with her eyes closed. The patient showed a mild wide-based gait. (MP4 44624 kb)

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Ueno, T., Nishizawa, H., Suzuki, C. et al. Downbeat nystagmus as an initial clinical sign in spinocerebellar ataxia type 6. Neurol Sci 38, 1543–1545 (2017). https://doi.org/10.1007/s10072-017-2973-y

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  • DOI: https://doi.org/10.1007/s10072-017-2973-y

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