Clinically atypical expression of pathologically typical Lewy-body parkinsonism

Six patients autopsied by the neuropathology service at UMDNJ-Robert Wood Johnson Medical School between 1985 and 1988 had pathologically typical Lewy-body Parkinson's disease (PD). Review of their clinical records revealed that none had clinically typical PD. Atypical clinical features included juvenile onset, retrocollis, strong family history, and absence of tremor, flexed posture, or levodopa response. One patient had dementia without parkinsonism. We conclude that the clinical spectrum of Lewy-body PD is wider than is generally assumed and that the diagnosis of pathologically typical Parkinson's disease cannot be excluded on clinical grounds alone.