THE DISTRIBUTION OF LEWY BODIES IN PURE AUTONOMIC FAILURE - AUTOPSY FINDINGS AND REVIEW OF THE LITERATURE

Pure autonomic failure (PAF; also known as idiopathic orthostatic hypo tension or Bradbury-Eggleston syndrome) is an uncommon sporadic disord er, characterized by autonomic failure without other neurological defi cits and histopathologically bf cell loss in intermediolateral columns and sympathetic ganglia. Few postmortem studies of patients With PAF have been reported in the literature, and none have demonstrated Lewy bodies in distal axons, although this has been described as a feature in Parkinson's disease with autonomic failure. We report a patient wit h PAF who had orthostatic hypotension and urinary symptoms for 15 year s prior to death at the age of 63 years. Postmortem findings included typical and atypical Lewy bodies in the substantia nigra, locus cerule us, substantia innominata, and sympathetic ganglia, as well as in auto nomic axons in the epicardial fat, autonomic nerve fascicles In periad renal adipose tissue, and autonomic nerves in the muscularis of the ur inary bladder Sites of autonomic nerve involvement correlated with cli nical symptomatology, and thus were a valuable observation In the comp lete autopsy. Systemic autopsy results should be reviewed carefully in patients with PAF, as Lewy bodies in this disease may be seen in dist al axons at a great length from their primary cell bodies.