A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970-1979. II: Epidemiology.

Abstract

A comprehensive search yielded 121 confirmed cases of Creutzfeldt-Jakob disease who died in England and Wales in the decade 1970-1979, 31 probable cases and 10 possible cases. Descriptive epidemiological data are presented. The average annual incidence was 0 3 cases/million. An unexpected female excess was found. There was no evidence of space-time clustering of cases and no associations with occupation or past medical treatment were apparent. There was statistically significant variation in incidence rates in different parts of the country but no relationship was discovered between incidence and population density. Creutzfeldt-Jakob disease was first transmitted to the chimpanzee in 1968,' but despite extensive subsequent investigation no means of natural transmission has been discovered. Lateral transmission of an agent is thought to occur in scrapie,2 transmissible mink encephalopathy,3 and kuru4 but, with the exception of rare cases of iatrogenic transmission and possible contact transmission with familial cases, has not been described in Creutzfeldt-Jakob disease. As part of an epidemiological survey of the disease in England and Wales a retrospective survey was undertaken for the decade 1970-1979. The present communication is concerned with the epidemiological aspects of the survey. Methods Cases were ascertained from three sources. The primary source of cases was from death certificates. The 8th ICD revision, which was in use between 1970-1978, did not have a separate code for Creutzfeldt-Jakob disease and it was first necessary to define rubrics likely to include patients dying of Creutzfeldt-Jakob disease. Death certificates for the year 1973 was available on microfilm at the Office of Population Censuses and Surveys, and all certificates coded under rubrics 348, 290-0, 290-1, 333.9, 347.9, 3319 and 342 were examined for mention of Creutzfeldt-Jakob disease or Subacute Spongiform Encephalopathy. (A list of rubrics is appended.) Two cases certified as Creutzfeldt-Jakob disease were coded under rubric 290-1 and twelve cases under rubric Address for reprint requests: RG Will,

Incidence of Creutzfeldt-Jakob disease in Taiwan: a prospective 10-year surveillance

“…The overall incidences remained in the range between 0.5 and 0.6 per million person year. And the incidence of patients with CJD onset after 80 years of age was still high without sharply decline, which was different from previous reports of other countries [20,21,[26][27][28]. The genetic study of the codon 129 of the PRNP genotype revealed that our patients were all Met/Met methionine homozygous instead of heterozygous.…”

Epidemiological surveillance of Creutzfeldt-Jakob disease in the United Kingdom

“…Age specific incidence rates, with the majority of cases in the 60-75 year age group, are consistent in national surveys, but the previously described female excess of cases in England and Wales (26,11) has been maintained in the current study. This female preponderance, also described in some other countries, is unexplained.…”

?Clusters? of CJD in Slovakia: The first statistically significant temporo-spatial accumulations of rural cases

“…Significant variations in CJD case rates were noted previously in France and in England (3,4,18,19). As possible explanations, ethnic compositions, environmental factors, case finding artifacts or case-to case transmisssion as a less probable eventuality, have been suggested (17).…”

“…Detailed statistical studies have been performed on CJD focal occurrence (2,3,10,17). Apart from familial CJD clustering, no statistically significant temporospatial relationships have been demonstrated up to now (4,18).…”