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Gerstmann-Sträussler-Scheinker disease (GSS)

Accordion Content
Gerstmann-Sträussler-Scheinker disease (GSS) is an extremely rare, neurodegenerative brain disorder caused by prion (proteins) that misfold in the brain, primarily in the cerebellum.

Typical early symptoms: ataxia (gait changes), balance problems, difficulty walking, incoordination. Sometimes Parkinson’s-like symptoms.

May also include: dysarthria (difficulty speaking), nystagmus (movement of the eyes), hearing problems, spasticity, visual disturbances including double vision, and dementia.

Genetic

At least 56 known families
2-10 years
Midlife: 30s, 40s, 50s
Clinical examination, family history, genetic testing, brain MRI, spinal fluid testing (RT-QuIC) (Contact the CJD Foundation for specifics)
There are several mutations that cause GSS. The most common mutations include P102L and A117V.

Resources:

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A teleconference with Michael Geschwind, M.D., Ph.D., UCSF

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This website was made possible by a generous donation from Cookie Stivison, in memory of her husband Tom Stivison, and a grant from the Centers for Disease Control and Prevention.