Prion diseases in pediatrics

Prion diseases are rare neurologic affections with a poor prognosis, occurr ing in both humans and animals. Creutzfeldt-Jakob disease (CJD) secondary t o human extracted growth hormone treatment is the most frequent condition i n pediatrics. In 1994 a new type of CJD (variant CID) was described in youn g adults in the United Kingdom only 10 years after the bovine spongiform en cephalopathy epidemic, with recent works showing a direct relationship betw een the bovine epidemic and the human cases. An accumulation of a single pr otein called the prion protein (PrP) has been discovered in the brain in al l of these cases, animal and human, leading to the hypothesis that a new in fectious agent could proceed without any nuclear acid information; another hypothesis is that of a still unknown viral agent. The PRNP gene encoding f or this PrP protein is well described: some mutations and a polymorphism in the 129th codon have been shown to be implicated in many cases of CJD. PrP is a ubiquitous protein, with yet unknown physiological function There are still many questions to be answered: shall we expect new pediatric cases o f variant CJD? Assuming that animal-human contamination is related to alime ntation, are there other ways of contamination? (C) 1999 Elsevier, Paris.