Gerstmann-Sträussler-Scheinker syndrome

From Freepedia

Gerstmann-Sträussler-Scheinker syndrome (GSS) is a very rare, usually familial, neurodegenerative disease that affects patients in the third to seventh decades of life. It is one of the few diseases which are caused by prions.

The exact incidence of GSS is unknown but is estimated to be between 1 to 10 per 100 million. Familial cases are associated with autosomal dominance inheritance in families. A change in codon 102 from proline to leucine was found in the PrP gene of affected individuals. Therefore, it appears this genetic change is essential for the acquisition of the disease.