A French cluster of Creutzfeldt-Jakob disease: A molecular analysis

P. Beaudry, P. Parchi, K. Peoc'h, P. Desbordes, J. F. Dartigues, A. Vital, C. Vital, S. Capellari, P. Gambetti, N. Delasnerie-Lauprêtre, J. Y. Mary, J. L. Laplanche

Research output: Contribution to journalArticle

Abstract

We report the molecular and phenotypic analysis of a French cluster of three cases of Creutzfeldt-Jakob disease (CJD), two of them occurring in 1998 in the same village and the other in 1995 in a neighboring village. Analyses of the occurrence of these events in a close area with less than 3000 inhabitants over the 1992-1999 notification period confirmed that they are rare. This could be explained either by a common source of contamination or by the coincidental occurrence of either sporadic or genetic CJD. We applied genetic analysis and brain PrPres typing to explore these CJD cases. The three patients did not carry any mutation in their prion protein gene coding sequence. All were homozygous for methionine at the polymorphic codon 129. Brain tissue was available from two cases that died in 1998. The two patients showed different PrPres profiles on Western blot and distinct clinico-pathological features. These findings do not support the conclusion that in these three cases, CJD was acquired from a unique source of contamination and suggest that concurrent occurrence of sporadic CJD accounted for this CJD cluster.

Original languageEnglish
Pages (from-to)457-462
Number of pages6
JournalEuropean Journal of Neurology
Volume9
Issue number5
DOIs
Publication statusPublished - 2002

Keywords

  • Cluster
  • Creutzfeldt-Jakob disease
  • Prion
  • Strain

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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    Beaudry, P., Parchi, P., Peoc'h, K., Desbordes, P., Dartigues, J. F., Vital, A., Vital, C., Capellari, S., Gambetti, P., Delasnerie-Lauprêtre, N., Mary, J. Y., & Laplanche, J. L. (2002). A French cluster of Creutzfeldt-Jakob disease: A molecular analysis. European Journal of Neurology, 9(5), 457-462. https://doi.org/10.1046/j.1468-1331.2002.00456.x