“Body-first” dementia with Lewy bodies

According to the synucleinopathy staging schema [1] and the brain-first/body-first theory [10], in Parkinson’s disease (PD) the pathogenetic process can begin outside the brain, with early involvement of the autonomic nervous system. The body-first concept might also apply to the closely related disease dementia with Lewy bodies (DLB) [11]. We report the case of a patient in whom DLB manifested clinically after he had been followed for several years as a participant in the prospective, longitudinal PDRisk study. He underwent comprehensive initial and serial assessments that enabled evaluation of the body-first sequence. It is important to confirm by postmortem analyses whether at-risk individuals who are diagnosed subsequently with DLB have supportive tissue neurochemical and neuropathologic evidence; however, such evidence is highly unusual. Here we report postmortem neurochemical and neuropathological data in the same patient. …