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 | Prions |
| Treatment | None |
| Medication | None |
| Prognosis | Universally fatal, life expectancy is typically 5-6 years from diagnosis |
| Frequency | 1-10 of every 100 million people |
Gerstmann–Sträussler–Scheinker syndrome (GSS) is an extremely rare, invariably fatal neurodegenerative disease that usually affects patients from 35 to 55 years in age. It is exclusively heritable, and is found in only a few families around the world. GSS is classified with the transmissible spongiform encephalopathies (TSE) due to the causative role played by PRNP, the human prion protein. It 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.