Definition:
Gerstmann-Sträussler-Scheinker syndrome (GSS) is a rare, deadly neurodegenerative disease that affects people between ages 20 and 60. GSS is classified as a transmissible spongiform encephalopathy (TSE).
Diagnosis:
Genetic testing is used to determine the onset of the mutations. A person's family history is also studied.
Treatment:
There is no treatment for the disease.
Symptoms and Signs:
The signs of the disease begin with slow development of speaking problems, ataxia and progressive dementia.
Causes:
GSS is caused by prions, pathogenic proteins that can resist proteases. It is a familial syndrome that shows mutations in the prion protein genes of affected persons.
