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Gerstmann-straussler-scheinker syndrome

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Gerstmann-straussler-scheinker syndrome

(Science: syndrome) a familial spongiform encephalopathy. Transgenic mice with a mutant form of the prp gene from patients with this syndrome develop degenerative brain disease that is similar, but not identical, to that caused by scrapie.

It is a more chronic cerebellar form of spongiform encephalopathy, producing a neurodegenerative condition that has morphological similarities to creutzfeldt-jakob syndrome. However, in this syndrome there is slower progression, signs of spinocerebellar ataxia, and the spongiosis is less pronounced.

prion proteins and amyloid plaques are found in the brain of patients with the syndrome. A germline mutation of the prion protein has been demonstrated.