Caused by the GSS prion, this disease was first described in 1928.
Gerstmann-Straussler-Scheinker disease is always fatal.
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.
There are no available treatments for Gerstmann-Straussler-Scheinker disease. It is relentlessly progressive, incurable, and fatal. Supportive care for the patient and his or her family is the only treatment.
Early symptoms include unsteady gait and difficulty walking, discoordination, clumsiness.
It is characterized by progressive cerebellar ataxia and associated motor complications, following a time course of 2-10 years before death.
Gerstmann-Sträussler-Scheinker disease (GSS) is a hereditary form of prion disease caused by mutations in the PRNP gene. It is characterized by progressive neurodegeneration, ataxia, and dementia. Symptoms typically manifest in adulthood.
Through characteristic abnormalities found on the electroencephalogram (EEG), a test of brain waves and electricity. MRI studies and biopsies (tissue samples) from the brain may also show changes that are characteristic of prion disease.
Human Prion DiseasesCreutzfeldt-Jakob Disease (CJD) Variant Creutzfeldt-Jakob Disease (vCJD)Gerstmann-Straussler-Scheinker SyndromeFatal Familial InsomniaKuru
Prions are infectious proteins. Examples are the well known "mad cow disease" or bovine spongiform encephalopathy (BSE; affects cattle), scrapie (affects sheep), Creutzfeldt-Jacob disease, Kuru, Gerstmann-Sträussler-Scheinker syndrome, and Fatal familial insomnia. Variant Creutzfeldt-Jakob disease (vCJD) is the disease that occurs in humans when BSE proteins from cattle enter humans.
my neighbor discovered it
it was discovered in wellington
it was discovered in wellington