IATROGENIC CREUTZFELDT-JAKOB-DISEASE IN 3 GROWTH-HORMONE RECIPIENTS -A NEUROPATHOLOGICAL STUDY

Since 1985, several cases of Creutzfeldt-Jakob disease, occurring afte r a treatment by human cadaveric hormone have been reported. Three new iatrogenic cases observed in French patients (two children and one yo ung adult) are described here. Neuropathological study displayed the c lassical aspects of previously reported sporadic cases of Creutzfeldt- Jakob disease in adults, including severe cortical spongiform change w ith numerous vacuoles within neuronal dendrites, diffuse astrogliosis and neuronal loss. In addition, the iatrogenic cases described here in cluded two more unusual points: (i) they were homozygotic for the PrP gene on codon 129 and therefore a genetic predisposition could be susp ected; (ii) numerous kuru plaques were scattered in the cerebral corte x, the subcortical white matter and in the cerebellar cortex. They wer e decorated with a PrP monoclonal antibody, but not with a beta A(4) a ntibody. This last point underlines the similarities between iatrogeni c Creutzfeldt-Jakob disease and kuru.