Abstract

Background: Pure autonomic failure (PAF) is a selective peripheral disorder in which Lewy bodies form within the autonomic ganglia. Patients with this disorder usually have no central lesions; however, chronic autonomic failure may secondarily affect the central nervous system. This study evaluated brain perfusion in patients with PAF by using N-isopropyl-p-¹²³I iodoamphetamine (¹²³I-IMP) single photon emission computed tomography (SPECT).

Methods: Six patients with PAF (all men; mean (SD) age 68±5 years) who had experienced autonomic symptoms for more than 5 years and six age-matched healthy control subjects (all men; mean (SD) age 67±5 years) were included in this study. The regions of interest (ROI) on spacially normalized ¹²³I-IMP SPECT images were automatically computed for both groups.

Results: Perfusion of the dorsal anterior cingulate cortex was decreased in the PAF group compared with the healthy control group (0.93 vs 1.01; p<0.001). In the other brain regions measured, there was no significant difference in regional perfusion between the two groups.

Conclusions: The dorsal anterior cingulate cortex is poorly perfused and may be functionally altered in patients with PAF. The reduced perfusion in such individuals may be a secondary change that results from chronic autonomic failure.