Acute autonomic neuropathies

Lindsay Zilliox MD (Dr. Zilliox of the University of Maryland Medical Center has no relevant financial relationships to disclose.)
James W Russell MD MS (Dr. Russell of the University of Maryland 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 6, 2002; last updated January 19, 2016; expires January 19, 2019

This article includes discussion of acute autonomic neuropathies, acute cholinergic neuropathy, and acute paraneoplastic autonomic neuropathy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The authors review the clinical presentation, diagnosis, pathogenesis, and management of acute autonomic neuropathies. Research indicates that many patients with acute autonomic neuropathy test positive for elevated ganglionic acetylcholine receptor autoantibodies and present with a syndrome complex including the sicca syndrome, orthostatic hypotension, lower gastrointestinal dysautonomia, and neurogenic bladder. Mouse models in which this antibody is induced also develop autonomic neuropathy. Other causes of acute autonomic neuropathy, including Guillain-Barré syndrome and paraneoplastic syndrome, are described. Many patients with autoimmune autonomic neuropathy respond rapidly and well to immune modulating therapy, including intravenous immunoglobulin. The management and treatment of these disorders is discussed, including information on the treatment of infant botulism.

Key points

 

• All acute autonomic neuropathies share common clinical features that may include orthostatic hypotension, dry eyes and mouth, cold feet and hands with color or trophic changes in the skin, reduced or accentuated sweating, anorexia or early satiety referable to gastroparesis, erectile or ejaculatory failure, and defects in micturition stream or volume, but selective syndromes are known.

 

• Autoantibodies to nicotine ganglionic acetylcholine receptors likely have a pathogenic role in autoimmune autonomic neuropathy.

 

• The presence of dysautonomia is associated with a higher incidence of mortality from Guillain-Barré syndrome.

 

• Assessment of a patient with a suspected autonomic neuropathy includes a detailed history, blood pressure and heart rate measurement with the patient supine and after standing for 1 minute, and laboratory tests of the autonomic nervous system (eg, tests of cardiovagal function, tests of sympathetic function, and testing for gastroparesis).

 

• Some autonomic neuropathies are amenable to treatment of the underlying disease, and autoimmune autonomic neuropathies may be responsive to intravenous gammaglobulin or other immunosuppressive treatments.

Historical note and terminology

Acute autonomic neuropathy is an inclusive term used to describe diseases resulting from distinct etiologies but that have in common pathology of the peripheral autonomic nervous system. Acute intermittent porphyria has been implicated in King George III of England's neuropsychiatric illness.

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