Pure autonomic failure

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Bradbury-Eggleston syndrome; idiopathic orthostatic hypotension; idiopathic hypotension

Overview

Pure autonomic failure is a form of dysautonomia

Classification

It is one of three diseases classified as primary autonomic failure.

Pathophysiology

The pathology of pure autonomic failure is not yet completely understood. However, a loss of cells in the intermediolateral column of the spinal cord has been documented, as has a loss of catecholamine uptake and catecholamine fluorescence in sympathetic postganglionic neurons. In general, levels of catecholamines in these patients are very low while lying down, and do not increase much upon standing.

Epidemiology and Demographics

First occurs in middle age or later in life; men are affected more often than women.

Diagnosis

Symptoms

A degenerative disease of the peripheral nervous system, symptoms include:

Common

• Dizziness
• Fainting or syncope (caused by orthostatic hypotension)
• Neck pain
• Visual disturbances

Less Common

Chest pain, fatigue and sexual dysfunction are less common symptoms that may also occur. Symptoms are worst when standing; sometimes one may relieve symptoms by laying down.

Treatment

Pharmacological methods of treatment include fludrocortisone, midodrine, somatostatin, erythropoietin, and other vasopressor agents. However, often a patient with pure autonomic failure can mitigate his or her symptoms with far less costly means. Compressing the legs and lower body, through crossing the legs, squatting, or the use of compression stockings can help. Also, ingesting more water than usual can increase blood pressure and relieve some symptoms.

External links

• National Dysautonomia Research Foundation (NDRF)
• Dysautonomia Support Network