Premium
Creutzfeldt‐Jakob disease in France: III. Epidemiological study of 170 Patients dying during the decade 1968–1977
Author(s) -
Brown Paul,
Cathala Francoise,
Gajdusek D. C.
Publication year - 1979
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410060511
Subject(s) - epidemiology , disease , medicine , creutzfeldt jakob syndrome , virology , pathology , prion protein
Systematic search for Creutzfeldt‐Jakob disease (CJD) in France yielded a total of 170 patients dying between 1968 and 1977. The overall annual mortality rate from CJD for France was 0.32 case per million persons, with by far the highest rate (1.33 cases per million) in the most densely populated parts of Paris. A highly significant correlation between population density and the mortality rate of CJD in the Paris urban agglomeration was consistent with the hypothesis of human‐to‐human disease transmissison, although in the rest of France CJD occurred at equally low rates among rural and urban populations. Temporospatial case clustering was not observed, nor could occupation, surgery, or potential exposure to scrapie be associated with an increased risk of contracting CJD. Six to 9% of cases were familial, analysis of which discouraged the notion of a familial “common exposure” in favor of genetic transmission of susceptibility to the virus or of the virus itself. A prospective epidemiological study of newly occurring cases of CJD, especially familial and geographically isolated cases, is being undertaken in the hope of identifying the means by which CJD is naturally acquired.