Creutzfeldt-Jakob disease in a husband and wife

P 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 journalArticlepeer-review

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
Pages (from-to)684-8
Number of pages5
JournalNeurology
Volume50
Issue number3
Publication statusPublished - Mar 1998

Keywords

  • Animals
  • Blotting, Western
  • Brain
  • Creutzfeldt-Jakob Syndrome
  • Cricetinae
  • Disease Transmission, Infectious
  • Drug Resistance
  • Endopeptidases
  • Family Health
  • Humans
  • Male
  • Middle Aged
  • Mutation
  • Prions
  • Reference Values
  • Spouses

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