Pure progressive autonomic failure: a clinicopathological study

Abstract

Two cases of pure progressive autonomic failure (PAF) are presented. A postmortem study of one case (case 2) showed a pathology resembling that of Parkinson's disease. Marked cell loss was noted in the substantia nigra, nucleus ceruleus, and intermediolateral column of the spinal cord, while cell loss in the sympathetic ganglion was not remarkable. This case may be an exceptionally rare case of late-onset PAF in which autonomic failure was mainly ascribed to preganglionic (and central) pathology, although autonomic function tests suggested postganglionic sympathetic disorder in both cases.