Chronic autonomic neuropathies

Mamatha Pasnoor MD (Dr. Pasnoor of the University of Kansas Medical Center has no relevant financial relationships to disclose.)
Mazen M Dimachkie MD (Dr. Dimachkie, Director of the Neuromuscular Disease Division and Vice Chairman for Research Programs, Department of Neurology, The University of Kansas Medical Center, received honorariums from Baxter, Genzyme, and Pfizer for serving as a guest speaker; consultation fees from Catalyst and Nufactor; and honorariums from Alnylam, CSL-Behring, Malinckrodt, and Novartis for advisory board meetings.)
Louis H Weimer MD, editor. (Dr. Weimer of Columbia University has received consulting fees from Roche.)
Originally released February 6, 2002; last updated January 7, 2016; expires January 7, 2019

This article includes discussion of chronic autonomic neuropathies, hereditary autonomic neuropathies, idiopathic distal small-fiber neuropathy, infective causes of autonomic neuropathy, metabolic and nutritional causes of autonomic neuropathy, pure cholinergic autonomic neuropathy, and toxic and drug-induced autonomic neuropathies. 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 chronic autonomic neuropathies. Autonomic neuropathies are acquired or inherited diseases resulting from a disturbance of the peripheral autonomic nervous system. New treatments have emerged for familial autonomic neuropathies, and droxidopa has also been approved for neurogenic orthostatic hypotension. Prion proteins have also been implicated in some forms of autonomic neuropathies.

Historical note and terminology

Chronic 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.

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