Creutzfeldt-Jakob disease in a husband and wife

Paul Brown, L. Cervenáková, L. McShane, L. G. Goldfarb, K. Bishop, F. Bastian, J. Kirkpatrick, P. Piccardo, B. Ghetti, D. C. Gajdusek

Research output: Contribution to journalArticle

31 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)684-688
Number of pages5
Issue number3
StatePublished - Mar 1998

ASJC Scopus subject areas

  • Clinical Neurology

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    Brown, P., Cervenáková, L., McShane, L., Goldfarb, L. G., Bishop, K., Bastian, F., Kirkpatrick, J., Piccardo, P., Ghetti, B., & Gajdusek, D. C. (1998). Creutzfeldt-Jakob disease in a husband and wife. Neurology, 50(3), 684-688.