Gerstmann–Sträussler–Scheinker syndrome

Gerstmann–Sträussler–Scheinker syndrome
Gerstmann–Sträussler–Scheinker syndrome
	A person with inherited prion disease has cerebellar atrophy. This is quite typical of GSS.
Specialty	Neurology
Symptoms	difficulty speaking, developing dementia, memory loss, vision loss.
Causes	prion
Prognosis	Universally fatal, life expectancy is typically 5-6 years from diagnosis

Gerstmann–Sträussler–Scheinker syndrome (GSS) is an extremely rare, always fatal (due to it being caused by prions) neurodegenerative disease that affects patients from 20 to 60 years in age. It is exclusively heritable, and is found in only a few families all over the world. It is, however, classified with the transmissible spongiform encephalopathies (TSE) due to the causative role played by PRNP, the human prion protein. GSS was first reported by the Austrian physicians Josef Gerstmann, Ernst Sträussler and Ilya Scheinker in 1936.

Familial cases are associated with autosomal-dominant inheritance.

Certain symptoms are common to GSS, such as progressive ataxia, pyramidal signs, and dementia; they worsen as the disease progresses.

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