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.
Human Prion DiseasesCreutzfeldt-Jakob Disease (CJD) Variant Creutzfeldt-Jakob Disease (vCJD)Gerstmann-Straussler-Scheinker SyndromeFatal Familial InsomniaKuru
It is characterized by progressive cerebellar ataxia and associated motor complications, following a time course of 2-10 years before death.
Prion diseases can also be hereditary, as seen in some cases of Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS).
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.
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
DEARBORN discovered this disease in 1932
it was discovered in wellington