NR ABJA
AU Billette de Villemeur,T.B.
TI [Juvenile form of Creutzfeldt-Jakob disease. Clinical and neuropathological aspects]
OT Forme juvenile de la maladie de Creutzfeldt-Jakob. Aspects cliniques et neuropathologiques.
QU Pathologie Biologie 1995 Feb; 43(2): 91-6
PT journal article; review; review literature
AB 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). Amyloid 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.
IN In diesem Review wird die Übertragung der Creutzfeldt-Jakob-Krankheit durch Bluttransfusion oder Blutübertragung auf Tiere erwähnt.
ZR 40
MH Adolescent; Adult; Aged; Child; Creutzfeldt-Jakob Syndrome/*etiology/pathology; English Abstract; Female; Human; Iatrogenic Disease; Male; Microscopy, Electron; Middle Age
AD Service de Neuropediatrie, Hopital Necker Enfants-Malades, Paris, France.
SP französisch
PO Frankreich