To investigate the characteristics of regional cerebral metabolism in a subgroup of patients with Parkinson's disease and autonomic failure, we studied seven patients with Parkinson's disease with autonomic failure (PA group), 11 patients with Parkinson's disease without apparent autonomic failure (PD group), and nine normal controls using fluoro-deoxyglucose positron emission tomography (FDG-PET). To determine differences in metabolic distribution among these groups, regional relative glucose metabolic rates (RGMR), which were normalized with cerebellar values, were calculated and age-adjusted covariance analyses were done. When compared with that of controls. RGMR in the cerebral cortex of the PA group was markedly reduced in the occipital cortex (P<0.001), inferior parietal cortex (P<0.005) and superior parietal cortex (P<0.005), but without a decrease in the sensory motor and medial temporal cortices, putamen and thalamus. In contrast, the PD group did not show significant focal hypometabolic distribution. Our findings raise the possibility that Parkinson's disease with autonomic failure may overlap with the features of dementia with Lewy bodies.