Gerstmann-Scheinker Syndrome (Gss)

(Gerstmann Scheinker syndrome)

Gerstmann-Straussler-Scheinker syndrome is an autosomal dominant prion brain disorder that typically begins in middle age.

The GSS occurs worldwide and is 100 times rarer than CJD. It develops at an earlier age (40 vs. 60 years), and the average life expectancy is longer (5 years vs. 6 months).

Gerstmann-Straussler-Scheinker syndrome is an autosomal dominant prion brain disorder that typically begins in middle age. The GSS occurs worldwide and is 100 times rarer than CJD. It develops at an earlier age (40 vs. 60 years), and the average life expectancy is longer (5 years vs. 6 months). The patients have cerebellar dysfunction unsteady, dysarthria and nystagmus. Looking paralysis, deafness, dementia, parkinsonism, hyporeflexia and a positive Babinski sign are also common. A myoclonus is much rarer than in the CJK. A GSS should be considered in patients with characteristic symptoms and signs as well as a family history, v. a. in an age of ? 45 years. A genetic test can confirm the diagnosis. There is only supportive care at GSS.

Health Life Media Team

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