Pure autonomic failure

The disorder now called pure autonomic failure (PAF) was first described by Bradbury and Eggleston1 in 1925. The original report included 3 patients with chronic postural hypotension and frequent episodes of syncope, a relatively slow and unchanging pulse rate, reduced sweating, erectile dysfunction, and constipation. Although the authors speculated that failure of the autonomic nervous system produced this syndrome, it was not until 1966 that Johnson et al.2 provided neuropathologic evidence for degeneration of sympathetic preganglionic neurons in this disorder. The first neuropathologic evidence for loss of sympathetic postganglionic neurons in PAF was reported by Goodall et al.3 and elegantly confirmed by Bannister et al.,4 who found virtual absence of catecholamine fluorescence in perivascular nerve plexuses in quadriceps muscle of 2 patients with PAF. Surprisingly, the patient with PAF reported by Johnson et al.2 (their patient 1) had typical intraneuronal Lewy bodies identical to those seen in patients with Parkinson disease (PD). This was not a coincidental finding. Following this report, several other cases of patients with PAF with detailed clinical histories and neuropathology confirmed the presence of Lewy bodies in sympathetic ganglia and distal sympathetic axons as well as in the substantia nigra or locus ceruleus. …