Sign Up for a Free Account
  • Updated 05.07.2022
  • Released 02.07.2007
  • Expires For CME 05.07.2025

Pure autonomic failure

Introduction

Overview

In this article, the author reports on the entity known as pure autonomic failure. The author details the clinical features, pathogenesis and pathophysiology, and the oftentimes difficult differential diagnosis. In addition, the author sheds light on developments, including predictive factors for phenotype conversion of patients with an earlier diagnosis of pure autonomic failure and more sinister diagnoses like multiple system atrophy or dementia with Lewy bodies.

Key points

• Pure autonomic failure remains a diagnosis of exclusion. However, newer biomarkers can assist in distinguishing between the development of Lewy body diseases and multiple system atrophy.

• Careful history taking, physical examination, and meticulous workup are needed to exclude other similar differential diagnoses.


• More sinister synucleinopathies, notably multiple system atrophy, often start with a similar clinical picture to pure autonomic failure.


• Specific attention should be directed toward some of the clinical features that were found to predict future development of multiple system atrophy or dementia with Lewy bodies in cases that are diagnosed initially with pure autonomic failure.

Historical note and terminology

In 1898, Langley coined the term “autonomic nervous system.” He identified the enteric, sympathetic, and parasympathetic components. Cannon later added the adrenal-hormonal component in the early 20th century.

In 1925, Samuel Bradbury and Cary Eggleston first described the entity now known as pure autonomic failure, which encompasses the failure of both the sympathetic and the parasympathetic nervous systems. Initially, these two clinicians described three patients with idiopathic orthostatic hypotension, which is the key characteristic of this uncommon disease (04). The nomenclature evolved from idiopathic orthostatic hypotension (Bradbury-Eggleston syndrome) into pure progressive autonomic failure and finally to the generally accepted term, pure autonomic failure. Some cardiologists still employ the term “idiopathic orthostatic hypotension.” The name pure autonomic failure was introduced by Oppenheimer as one of the primary chronic autonomic failure syndromes in addition to Parkinson disease with autonomic failure and multiple system atrophy.

Pure autonomic failure was defined by the 1996 Consensus Committee of the American Autonomic Society and the American Academy of Neurology to be “an idiopathic sporadic disorder characterized by orthostatic hypotension usually with evidence of more widespread autonomic failure and no other neurological features” (08).

This is an article preview.
Start a Free Account
to access the full version.

  • Nearly 3,000 illustrations, including video clips of neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of neurology in 1,200 comprehensive articles.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com

ISSN: 2831-9125