Sub-Acute Spongiform Encephalopathy With Multiform Plaque-Formation - Peculiar Familial Hereditary-Disease Of Cns [Spinocerebellar Atrophy With Dementia, Plaques, And Plaque-Like Deposits In Cerebellum And Cerebrum (Gerstmann, Straussler, Scheinker)]

Report of two unrelated cases of a rare familiar disease of degenerative nature, from a clinical point of view belonging to the spinocerebellar atrophies, combined with dementia. According to the pedigrees, the disease can be followed up to 3--4 generations. Microscopic study reveals glioneuronal dystrophy with spongiform changes together with kuru plaques and atypical plaque-like formations, hitherto only described in this disease. Nosological aspects of these findings are discussed.