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Gerstmann-Sträussler-Scheinker syndrome

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A rare prion disease which is a familial form of Creutzfeldt-Jakob syndrome. A widespread degeneration of the nervous system, starting usually in the fourth or fifth decade of life with slowly developing dysarthria and cerebellar ataxia and then dementia, accompanied by spinocerebellar and corticospinal tract degeneration. Absence of leg reflexes. Death follows 2 to 10 years after the onset of symptoms. Occurrence is one in a million people a year. Autosomal dominant inheritance.


  • J. Gerstmann, E. Sträussler, I. Scheinker:
    Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems. Zugleich ein Beitrag zur Frage des vorzeitigen lokalen Alterns.
    Zeitschrift für die gesamte Neurologie und Psychiatrie, 1936, 154: 736-762.

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