Maladie de Creutzfeld-Jakob unilatérale : à propos d'un cas probable sur les critères électrocliniques, IRM et biologiques

We report the case of a 70-year-old man who developed probable unilateral Creutzfeldt-Jakob disease. Clinically, he presented with right hemiparesis, progressive aphasia, temporospatial disorientation and cerebellar ataxia and later on, myoclonia. The MRI showed a hypersignal from the left caudate in DWI with decreased ADC. Repeated electroencephalograms showed a slow background rhythm in the left hemisphere with superimposed periodic, biphasic and triphasic sharp-wave complexes in the left temporal region. Death occurred after 5 weeks. Although exceptional, unilateral Creutzfeldt-Jakob disease was retained as possible.