All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Gerstmann-Straussler-Schenker syndrome.
Medical expert of the article
Last reviewed: 04.07.2025
Gerstmann-Straussler-Schenker syndrome is an autosomal dominant prion disease that begins in middle age.
Epidemiology
Gerstmann-Straussler-Schenker syndrome is common and is similar to Creutzfeldt-Jakob disease, but the incidence of this syndrome is approximately 100 times lower than that of Creutzfeldt-Jakob disease. The disease develops at a younger age (40 years compared to 60), and the average life expectancy after onset of the disease is longer than that of Creutzfeldt-Jakob disease (5 years compared to 6 months).
Symptoms Herstmann-Straussler-Schenker syndrome.
Patients develop cerebellar ataxia, dysarthria, and nystagmus. Gaze paresis, deafness, dementia, hyporeflexia, and abnormal plantar reflexes may occur. Myoclonic seizures are much less common than in Creutzfeldt-Jakob disease. In the presence of characteristic symptoms and a family history in young people (under 45 years), Gerstmann-Straussler-Schenker syndrome is more likely.
[ 4 ]
Who to contact?