Creutzfeldt-Jakob disease in a husband and wife.
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.
Brown
P
P
Laboratory of CNS Studies, NINDS, National Institutes of Health, Bethesda, MD 20892, USA.
Cervenáková
L
L
McShane
L
L
Goldfarb
L G
LG
Bishop
K
K
Bastian
F
F
Kirkpatrick
J
J
Piccardo
P
P
Ghetti
B
B
Gajdusek
D C
DC
D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics / Q000378:metabolism / Q000635:transmission*; D006224:Cricetinae; D018562:Disease Transmission, Infectious; D004351:Drug Resistance; D010450:Endopeptidases / Q000494:pharmacology; D005192:Family Health; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011328:Prions / Q000187:drug effects / Q000235:genetics / Q000378:metabolism; D012016:Reference Values; D018454:Spouses
D011328:Prions; D010450:Endopeptidases