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The sporadic
form of CJD has an extremely low incidence, about one case per
million person-years, all over the world.
The etiology
of sporadic CJD remains unknown, and there is concern about the
possibility that some unrecognized factors may enhance the risk of
developing CJD. However, no evidence was found for any environmental
risk factor, so far.
CJD has been
accidentally transmitted by iatrogenic routes: the incubation
time is long, and highly variable between individuals: a mean
incubation time of 9 to 10 years, with a 95th percentile of the
distribution of 15 to 16 years, has been reported in French growth
hormone recipients who were homozygotes at codon 129 of the prion
protein gene (Huillard d'Aignaux J et al. Neurol 1999;53:1197-201).
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