Prise en charge des suspicions de maladie de Creutzfeldt-Jakob en réanimation

Abstract

Creutzfeldt-Jakob disease is a neurodegenerative disease whose main characteristic is dementia. It has three epidemiological forms: the main one, sporadic, the familial form associated with mutation of the gene encoding the prion protein and iatrogenic forms. This transmissible spongiform subacute encephalopathy is a rare disease with an incidence of one case per million inhabitants per year. The disease’s neuropathological characteristics are the presence of spongiosis, neuronal death and gliosis and the accumulation of the host-encoded protein, partly resistant to proteases. The new variant of Creutzfeldt-Jakob disease was described in Great-Britain in 1996 in young patients with no medical history to suggest a iatrogenic or familial origin. Hyperdense signals to cerebral MRI have been described in post-thalamic areas and can be a helpful diagnostic feature in cases with a suggestive clinical picture. This form was quickly suspected to be the consequence of infection in man with the bovine spongiform encephalopathy agent. To date, 130 cases have been recorded. The agent distribution in humans is largely peripheral. The safety of transplants, drugs, transfusion and regulations on the safety of hospitals need to be reassessed. From public health point of view, it is essential that all cases are identified.