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Holmes' syndrome I

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Inheritable disease picture with cerebellar ataxia due to a degeneration of cerebellum olivary nucleus. Progressive disturbance of gait, eventual uncertainty of movements of arms, speech changes (hesitant scanning and explosive). Later, cerebellar tremor and nystagmus. Mild hyperreflexia; no ankle clonus. Prevalent in males; onset gradual in sixth or seventh decade. Etiology unknown. Inheritance is autosomal dominant.


  • G. M. Holmes:
    A form of familial degeneration of the cerebellum.
    Brain, Oxford, 1907, 30: 466-489.

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