[Acquired forms of Creutzfeldt-Jakob disease].

AIM: To establish the recent neuropathological data concerning the acquired forms of human prions diseases at the exception of the new variant.

RESULTS

Kuru transmitted by cannibalism involved mainly the cerebellum with cerebellar tracts degeneration and presence of amyloid plaques called "kuru plaques" labeled by anti PrP antibodies. To day the disease has disappeared. Iatrogenic forms have been found after corneal graft or more frequently dura-mater graft. In these later cases, neuronal loss, spongiosis and gliosis are diffuse in cerebral and cerebellar cortex with the same intensity. The cases associated with pituitary-derived human growth hormone administration are observed in young patients. Histologic lesions, in 16 cases, are present in basal ganglia, thalamus, brain stem and predominantly in the cerebellum especially in the granule cell layer. Cortical involvement is moderate. Plaques have not been always looked for.

CONCLUSION

The diversity of acquired forms is in favor of the hypothesis of selection of specific strains.