Gerstmann straussler scheinker syndrome
(Science: syndrome) a familial spongiform encephalopathy. Transgenic mice with a mutant form of the prp gene from patients with this syndrome develop degenerative brain disease that is similar, but not identical, to that caused by scrapie.
It is a more chronic cerebellar form of spongiform encephalopathy, producing a neurodegenerative condition that has morphological similarities to creutzfeldt-jakob syndrome. However, in this syndrome there is slower progression, signs of spinocerebellar ataxia, and the spongiosis is less pronounced.