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Gerstmann-Sträussler-Scheinker Disease (GSS)

By

Pierluigi Gambetti

, MD, Case Western Reserve University

Last full review/revision Jul 2020| Content last modified Jul 2020
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Gerstmann-Sträussler-Scheinker disease is an autosomal dominant prion brain disease that typically begins during middle age.

Gerstmann-Sträussler-Scheinker disease (GSS) occurs worldwide and is about 100-fold less common than Creutzfeldt-Jakob disease Creutzfeldt-Jakob Disease (CJD) Creutzfeldt-Jakob disease (CJD) is the most common human prion disease. It occurs worldwide and has several forms and subtypes. CJD symptoms include dementia, myoclonus, and other central nervous... read more (CJD). It develops at an earlier age (40 versus 60 years), and average life expectancy is longer (5 years versus 6 months).

GSS should be considered in patients with characteristic symptoms and signs and a family history, particularly if they are 45 years. Genetic testing can confirm the diagnosis.

There is only supportive treatment for GSS.

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