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Marie's ataxia

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A hereditary disease of the nervous system, with cerebellar ataxia caused by bilateral cortical atrophy of the cerebellum. Both sexes affected. Onset in early adult life. Characteristic symptoms are spastic ataxic gait, poor coordination, static tremor, exaggerated tendon reflexes, impaired deep sensibility, pain, incoordination, intense tremor, and ataxia. The patient stands on a broad base, sways with the eyes open or closed, has severe nystagmuss on any attempted motion, and frequently presents oscillation or tremor of the head. Ophthalmic abnormalities include decreased central vision. Defects in the visual fields, and absent pupillary reflexes, which resembles the Argyll Robertson’s pupil. No skeletal deformities. Mental deterioration and sphincter disorders may occur in advanced stages.

Considered similar or identical to Dejerine’s and Friedreich’s ataxia. Inheritance is assumed to be autosomal dominant


  • M. Nonne:
    Über eine eigentümliche familiäre Erkrankungsform des Zentralnervensystems.
    Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1891, 22: 283-316.
  • P. Menzel:
    Beiträge zur Kenntnis der hereditären Ataxie und Kleinhirnatrophie.
    Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1891, 22: 160-190.
  • Sanger Brown:
    On hereditary ataxia, with a series of twenty-one cases.
    Brain, Oxford, 1892, 15: 250-282.
  • P. Marie:
    Sur l'hérédo-ataxie cérébelleuse.
    Clinique des maladies nerveuses. La semaine médicale, Paris, 1893, 13: 444-447.

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