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Spongiform encephalopathy
Gerstmann Straussler Scheinker syndromeGerstmann-Straussler-Scheinker diseaseAmyloidosis cerebral with spongiform encephalopathy
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Overview
Gerstmann-Straussler-Scheinker disease is a neurodegenerative brain disorder which causes ataxia and dementia. It is almost always inherited and is found in only a few families around the world. Onset of the disease usually occurs between the ages of 35 and 55. Gerstmann-Straussler-Scheinker disease belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or prion diseases. Other TSEs include Creutzfeldt-Jakob disease, kuru, and fatal familial insomnia.
Source: Genetic and Rare Diseases Information Center (GARD), supported by ORDR-NCATS and NHGRI.
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Genetic & Rare Diseases Information Center (GARD)
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