Phenotypic similarities causing clinical misdiagnosis of pathologically-confirmed sporadic Creutzfeldt-Jakob disease as dementia with Lewy bodies

A patient fulfilling central, core and supportive clinical diagnostic criteria for dementia with Lewy bodies deteriorated rapidly in the absence of neuroleptic drug treatment, prompting suspicion of a diagnosis of sporadic Creutzfeldt-Jakob disease. At postmortem examination, the brain showed features typical of Creutzfeldt-Jakob disease of the MV1 subtype. We review the phenotypic overlap between dementia with Lewy bodies and Creutzfeldt-Jakob disease which may cause clinical misdiagnosis.