[Case of Creutzfeldt-Jakob syndrome with involvement of the neostriatum].

The authors describe a case of CJD in a man aged 45 years. The disease began with sluggishness of movements and speech difficulties, followed by development of pyramidal system damage and dementia. EEG findings were normal throughout the whole duration of the disease that is 3.5 years. Neuropathological examination disclosed major neuronal loss, spongy degeneration and astrogliosis in the striatum bilaterally. The cerebral and cerebellar cortex and brain stem showed similar but much less intense changes. This particular topography of the lesions was probably the cause of absent EEG changes.