Also Known As
Gerstmann-Straussler-Scheinker diseaseprion dementiasubacute spongiform encephalopathy, Gerstmann-Straussler typeGSDGerstmann Straussler Scheinker syndromeGerstmann-Straussler diseaseamyloidosis cerebral with spongiform encephalopathyamyloidosis, cerebral, with spongiform encephalopathycerebellar ataxia, progressive dementia, and amyloid deposits in CNScerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous systemcerebral amyloid angiopathy, Prnp-relatedencephalopathy subacute spongiform Gerstmann-Straussler typeencephalopathy, Subacute spongiform, Gerstmann-Straussler type
Definition
A very rare and fatal disorder of spongiform encephalopathy usually caused by mutations of the prion protein (PRNP) gene. It is characterized by the accumulation of amyloid in the brain. Signs and symptoms include lack of motor coordination, unsteady gait, and difficulty walking. As the disease progresses, patients develop speech difficulties and dementia.