Autonomic Disorders Consortium

Information for Professionals

Disorders In Depth

Pure Autonomic Failure (PAF)

Introduction

Pure autonomic failure (PAF), or Bradbury-Eggleston syndrome, is a peripheral degenerative disorder of the autonomic nervous system presenting in middle to late life, and affecting men more often than women. The disorder appears to be confined to the sympathetic and parasympathetic nervous systems.

Clinical Features

Pathology

There is a loss of cells in the intermediolateral column of the spinal cord and a loss of catecholamine uptake and catecholamine fluorescence in sympathetic postganglionic neurons. Autonomic failure should be distinguished from two other disorders classified as primary autonomic failure: Multiple System Atrophy (MSA) and idiopathic Parkinson’s Disease (PD). PAF generally induces fewer disabling symptoms than do these other syndromes. In PAF, there should be no indication from the history or physical examination of cerebellar, striatal, pyramidal or extrapyramidal dysfunction. Autonomic failure patients have greatly reduced levels of catecholamines and an impaired response to stimuli that normally raise plasma norepinephrine and DHPG. Plasma and urinary norepinephrine levels are sometimes only 10% of normal. There is marked hypersensitivity to all pressor and depressor stimuli, especially sympathomimetic amines.

Treatment

Outlook

Patients with PAF have a generally good outlook; many live normal life spans and sometimes well into their 80s.