Trends in mortality from sporadic Creutzfeldt-Jakob disease in France 1992-7

This study examined trends in mortality from sporadic Creutzfeldt-Jakob dis ease in France for 1992-7 by age, genotype at the codon 129 of the prion pr otein gene, and geographical area. Case ascertainment was based on notifica tions by neurologists, neuropathologists, and laboratories; 324 deaths from definite or probable Creutzfeldt-Jakob disease were registered during the study period. The yearly number of deaths increased significantly between 1 992 and 1997. The rise was higher for older age groups. It was also higher in those who were homozygous for valine compared with other genotypes. Eigh teen departments (geographical administrative areas) out of 95 showed a sig nificant increase in the number of deaths from sporadic Creutzfeldt-Jakob d isease. Intensive epidemiological surveillance is a likely explanation for the apparent increase in the number of sporadic cases of Creutzfeldt-Jakob disease over the 1992-7 period, particularly in older age groups and in dep artments with low mortality rate at the beginning of the study period. Inte nsive surveillance may also have led to a better identification of atypical valine homozygous cases.