Definition

Gerstmann-Straussler-Scheinker disease is a progressively disabling and ultimately fatal brain infection caused by a unique protein particle called a prion. Gerstmann-Straussler-Scheinker disease is an inherited disorder, and occurs in familial clusters.

Description

Gerstmann-Straussler-Scheinker disease belongs to a group of diseases originally known as slow virus infections. Currently, slow virus infections are classed together as transmissible spongiform encephalopathies (TSE), or prion diseases. Other TSEs include kuru, Creutzfeldt-Jakob disease, and fatal familial insomnia. The TSE called new variant Creutzfeldt-Jakob disease (also known...