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Gerstmann-Straussler-Scheinker syndrome


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An autosomal dominant condition that is caused by a mutation in the prion protein gene and resembles Creutzfeldt-Jakob disease (CJD). Patients present with cerebellar dysfunction (ataxia and dysarthria) and later develop dementia. They continue to deteriorate over several years, in contrast with patients with CJD, who deteriorate rapidly over periods of less than 12 months. J. G. Gerstmann


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