[Juvenile form of Creutzfeldt-Jakob disease. Clinical and neuropathological aspects]

Abstract

Creutzfeldt-Jakob disease in young people is frequently iatrogenic due to growth hormone cadaveric treatment. Ataxia and diplopia are the main symptoms at onset, and the absence of dementia and of neuroradiological and biological abnormalities is striking. The proof of the disease is given by histological examination (spongiosis, neuronal loss, astroglial proliferation, no inflammatory signs). Amyloïd plaques are frequent in these iatrogenic cases. PrP storage in brain is recognised by immunohistochemistry and western-blot. Transmission to animals proves the power of the disease to be transmitted. The transmission by inoculation of a buffy coat from one patient and the recorded cases after blood transfusion justify it is necessary to exclude extractive growth hormone recipients from blood and organ donors.