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Gerstmann-Straussler-Schenker Syndrome
Last reviewed: 23.04.2024
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The Gerstmann-Straussler-Schenker syndrome is an autosomal dominant prion disease that begins in middle age.
Epidemiology
The Gerstmann-Straussler-Schenker syndrome is ubiquitous and similar to Creutzfeldt-Jakob disease, but the incidence of this syndrome is about 100 times lower than the incidence of Creutzfeldt-Jakob disease. The disease develops at a younger age (40 years compared to 60), and the average life expectancy after the debut of the disease exceeds that of Creutzfeldt-Jakob disease (5 years compared to 6 months).
Symptoms of the gerstmann-Straussler-Schenker syndrome
Patients develop cerebellar ataxia, dysarthria and nystagmus. Paresis of the eye, deafness, dementia, hyporeflexia and pathological plantar reflexes may join. Myoclonic cramps are much less common than with Creutzfeldt-Jakob disease. In the presence of characteristic symptoms and family history in young people (up to 45 years) is more likely the syndrome Gerstmann-Straussler-Schenker.
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