<unk>  (GSS) is an extremely rare, usually familial, fatal 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<unk>  and Ilya<unk>  in 1936. Familial cases are associated with autosomal-dominant inheritance. Certain symptoms are common to GSS, such as progressive ataxia, pyramidal signs, and even adult-onset dementia; they progress more as the disease progresses.
Gerstmann–Sträussler–Scheinker syndrome
Scheie syndrome
Hapnes Boman Skeie syndrome
Ruzicka Goerz Anton syndrome
Qazi–Markouizos syndrome
Hurler–Scheie syndrome
Zieve's syndrome
Gerstmann syndrome