Gerstmann-Straussler-Scheinker syndrome

Gerstmann-Straussler-Scheinker disease (GSS) is a type of Prion Disease. Prion diseases are a group of conditions that affect the nervous system. The main feature of GSS is a progressive degeneration of the cerebellum (a part of the brain that controls coordination, balance, equilibrium and muscle tone), as well as different degrees of dementia. Signs and symptoms generally develop between ages 35 and 50 years and may include weakness in the legs, poor reflexes, abnormal sensations, progressive ataxia, cognitive dysfunction, slurred speech, and spasticity. GSS is an extremely rare condition. 

General Readings

https://rarediseases.info.nih.gov/diseases/7690/gerstmann-straussler-scheinker-disease

https://www.ninds.nih.gov/Disorders/All-Disorders/Gerstmann-Straussler-Scheinker-Disease-Information-Page

Clinical Research

A Case of Sporadic Creutzfeldt–Jacob Disease with Unusually Long Survival: Correlation with Clinical Features, Electroencephalogram, and Brain Magnetic Resonance Imaging

Structural and functional neuroimaging in human prion diseases