Pure autonomic failure

Ataf Chaudry MD (Dr. Chaudry of Creighton University has no relevant financial relationships to disclose.)
Faisal Khan MD (Dr. Khan of Duke University has no relevant financial relationships to disclose.)
Pariwat Thaisetthawatkul MD (Dr. Thaisetthawatkul of the University of Nebraska has no relevant financial relationships to disclose.)
Louis H Weimer MD, editor. (Dr. Weimer of Columbia University has received consulting fees from Roche.)
Originally released February 7, 2007; last updated February 10, 2010; expires February 10, 2013
Notice: This article has expired and is therefore not available for CME credit.

This article includes discussion of pure autonomic failure, Bradbury-Eggleston syndrome, Bradbury Eggleston syndrome, pure progressive autonomic failure, and idiopathic orthostatic hypotension. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The authors report on the entity known as pure autonomic failure. The authors detail the clinical features, pathogenesis and pathophysiology, and the sometimes difficult differential diagnosis. In addition, they shed light on recent developments including possible future management with plasma exchange.

Historical note and terminology

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

It was in 1925 that 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 (Bradbury 1925). Initially, these 2 clinicians described 3 patients with idiopathic orthostatic hypotension, which is the key characteristic of this uncommon disease. 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. 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” (Consensus Committee of the American Autonomic Society and the American Academy of Neurology 1996).

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