neurologic disorders
Gerstmann Sträussler Scheinker Disease (GSS)
Introduction
Gerstmann-Sträussler-Scheinker disease is an autosomal dominant prion brain disease that typically begins during middle age. (See also Overview of Prion Diseases .) Gerstmann-Sträussler-Scheinker disease (GSS) occurs worldwide and is about 100-fold less common than Creutzfeldt-Jakob Disease (CJD) (CJD). It develops at an earlier age (40 versus 60 years), and average life expectancy is longer (5 years versus 6 months). Patients have Cerebellar Disorders with unsteady gait, dysarthria, and nystagmus. Gaze palsies, deafness, dementia, GSS should be considered in patients with characteristic symptoms and signs and a family history, particularly if they are≤45 years. Genetic testing can confirm the diagnosis. There is only supportive treatment for GSS. 