Dementia with Lewy bodies in a neuropathologic series of suspected Creutzfeldt-Jakob disease

Discriminating Creutzfeldt-Jakob disease (CJD) from dementia with Lewy bodi es (DLB) may be clinically difficult to achieve. The authors describe 10 pa tients with DLB initially referred to the French Network of Human Spongifor m Encephalopathies as having suspected CJD. In a series of 465 autopsied ca ses, DLB ranked second among degenerative alternative diagnoses to CJD. The authors analyzed the factors that contributed to misleading the diagnosis, and suggest that the detection of 14-3-3 protein in CSF may be useful to d istinguish CJD from DLB.