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

30 Citations (Scopus)

Abstract

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
JournalNeurology
Volume50
Issue number3
DOIs
StatePublished - Mar 1998

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Creutzfeldt-Jakob Syndrome
Spouses
Amyloidogenic Proteins
Widowhood
Nervous System Diseases
Molecular Biology
Peptide Hydrolases
Brain
Genes
Sporadic Creutzfeldt-Jakob Disease
Prion Proteins

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Brown, P., Cervenáková, L., McShane, L., Goldfarb, L. G., Bishop, K., Bastian, F., ... Gajdusek, D. C. (1998). Creutzfeldt-Jakob disease in a husband and wife. Neurology, 50(3), 684-688. https://doi.org/10.1212/WNL.50.3.684

Creutzfeldt-Jakob disease in a husband and wife. / Brown, Paul; Cervenáková, L.; McShane, L.; Goldfarb, L. G.; Bishop, K.; Bastian, F.; Kirkpatrick, J.; Piccardo, P.; Ghetti, B.; Gajdusek, D. C.

In: Neurology, Vol. 50, No. 3, 03.1998, p. 684-688.

Research output: Contribution to journalArticle

Brown, P, Cervenáková, L, McShane, L, Goldfarb, LG, Bishop, K, Bastian, F, Kirkpatrick, J, Piccardo, P, Ghetti, B & Gajdusek, DC 1998, 'Creutzfeldt-Jakob disease in a husband and wife', Neurology, vol. 50, no. 3, pp. 684-688. https://doi.org/10.1212/WNL.50.3.684
Brown P, Cervenáková L, McShane L, Goldfarb LG, Bishop K, Bastian F et al. Creutzfeldt-Jakob disease in a husband and wife. Neurology. 1998 Mar;50(3):684-688. https://doi.org/10.1212/WNL.50.3.684
Brown, Paul ; Cervenáková, L. ; McShane, L. ; Goldfarb, L. G. ; Bishop, K. ; Bastian, F. ; Kirkpatrick, J. ; Piccardo, P. ; Ghetti, B. ; Gajdusek, D. C. / Creutzfeldt-Jakob disease in a husband and wife. In: Neurology. 1998 ; Vol. 50, No. 3. pp. 684-688.
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