NR ABLA
AU Boellaard,J.W.; Schlote,W.
TI [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)]
OT Subakute spongiforme Encephalopathie mit multiformer Plaquebildung. "Eigenartige familiar-hereditare Krankheit des Zentralnervensystems [spino-cerebellare Atrophie mit Demenz, Plaques und plaqueähnlichen Ablagerungen im Klein- und Grossirn" (Gerstmann, Sträussler, Scheinker)].
QU Acta Neuropathologica 1980; 49(3): 205-12
PT journal article
AB 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.
MH Atrophy; Brain Diseases/*genetics/pathology; Case Report; Cerebellum/pathology; English Abstract; Female; Human; Middle Age; Neuroglia/pathology; Neurons/pathology; Pedigree; Spinal Cord/pathology
SP deutsch
PO Westdeutschland