NR ABVB
AU Brown,P.; Kaur,P.; Sulima,M.P.; Goldfarb,L.G.; Gibbs,C.J.Jr.; Gajdusek,D.C.
TI Real and imagined clinicopathological limits of "prion dementia"
QU Lancet 1993 Jan 16; 341(8838): 127-9
KI Lancet. 1993 Mar 6; 341(8845): 626-7; discussion 627-8. PMID: 8094846 Lancet. 1993 Mar 6; 341(8845): 626; discussion 627-8. PMID: 8094845 Lancet. 1993 Mar 6; 341(8845): 627; discussion 627-8. PMID: 8094847
PT journal article
AB The term "prion dementia" has been proposed to replace "spongiform encephalopathy", to accommodate the existence of atypical forms of these "prion protein" (PrP) cerebral amyloidoses that may not show spongiform changes in the brain. We tested brain tissue extracts for the presence of PrP from 46 cases (including 13 familial cases) of non-spongiform dementias with a variety of associated neurological signs, referred to our laboratory for primate transmission studies. None of the cases transmitted disease to primates, and none had PrP detectable by western immunoblots of extracted brain tissue. We conclude that prion dementias are not lurking undetected within the larger landscape of neurodegenerative disorders, and that their clinicopathological limits are, except for a small number of previously reported familial cases, essentially those of spongiform encephalopathy.
MH Adult; Aged; Animal; *Brain Chemistry; Cebidae; Cercopithecidae; Creutzfeldt-Jakob Syndrome/pathology/transmission; Dementia/*etiology/genetics/pathology; Female; Human; Male; Middle Age; Pan troglodytes; Prion Diseases/*etiology/genetics/pathology; Prions/*analysis
AD Paul Brown (pwb@codon.nih.gov), Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
SP englisch
PO England