[Subacute 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, Sträussler, Scheinker)] (author's transl)].
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.
D001284:Atrophy; D001927:Brain Diseases / Q000235:genetics* / Q000473:pathology; D002531:Cerebellum / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D009457:Neuroglia / Q000473:pathology; D009474:Neurons / Q000473:pathology; D010375:Pedigree; D013116:Spinal Cord / Q000473:pathology
Subakute spongiforme Encephalopathie mit multiformer Plaquebildung. "Eigenartige familiär-hereditäre Krankheit des Zentralnervensystems [spino-cerebellare Atrophie mit Demenz, Plaques und plaqueähnlichen Ablagerungen im Klein- und Grossirn" (Gerstmann, Sträussler, Scheinker)].