NR ABUP
AU Brown,P.; Cervenakova,L.; McShane,L.; Goldfarb,L.G.; Bishop,K.; Bastian,F.O.; Kirkpatrick,J.; Piccardo,P.; Ghetti,B.; Gajdusek,D.C.
TI Creutzfeldt-Jakob disease in a husband and wife
QU Neurology 1998 Mar; 50(3): 684-8
PT journal article
AB A 53-year-old man died of sporadic Creutzfeldt-Jakob disease (CJD) after a 1.5-year clinical course. Four and a half years later, his then 55-year-old widow died from CJD after a 1-month illness. Both patients had typical clinical and neuropathologic features of the disease, and pathognomonic proteinase-resistant amyloid protein ("prion" protein, or PrP) was present in both brains. Neither patient had a family history of neurologic disease, and molecular genetic analysis of their PrP genes was normal. No medical, surgical, or dietary antecedent of CJD was identified; therefore, we are left with the unanswerable alternatives of human-to-human transmission or the chance occurrence of sporadic CJD in a husband and wife.
IN Eine 55 Jahre alte Frau starb an der Creutzfeldt-Jakob-Krankheit, 4,5 Jahre nachdem bereits ihr damals 53 Jahre alter Ehemann ebenfalls an der Creutzfeldt-Jakob-Krankheit gestorben war. Beide hatten keine bekannten CJD-Fälle in ihren Familien und keine Hinweise auf mögliche iatrogene Infektionen. Bei dem 53-jährigen dauerte die klinische Phase seiner Creutzfeldt-Jakob-Krankheit 1,5 Jahre, bei seiner 55-jährigen Ehefrau dauerte die klinische Phase 1 Monat.
ZR 11
MH Animal; Blotting, Western; Brain/metabolism; Case Report; Creutzfeldt-Jakob Syndrome/genetics/metabolism/*transmission; Disease Transmission, Horizontal; Drug Resistance; Endopeptidases/pharmacology; Family Health; Hamsters; Human; Male; Middle Age; Mutation; Prions/drug effects/genetics/metabolism; Reference Values; *Spouses
AD Paul Brown (pwb@codon.nih.gov), Laboratory of CNS Studies, NINDS, National Institutes of Health, Bethesda, MD 20892, USA
SP englisch
PO USA