Dr. Dimachkie, Director of the Neuromuscular Disease Division and Executive Vice Chairman for Research Programs, Department of Neurology, The University of Kansas Medical Center, received honorariums from Alnylam, Audentes, Baxalta, Catalyst, CSL Behring, Mallinckrodt, Momemta, Novartis, NuFactor, Sanofi, Shire, RMS Medical, and Terumo for speaking engagements or consulting work, and grants from Alexion, Alnylam, Amicus, Biomarin, Catalyst, CSL Behring, FDA/OPD, Genentech, GlaxoSmithKline, Grifols, MDA Pharmaceuticals, NIH, Novartis, Octapharma, Orphazyme, Sanofi, UCB BioPharma, and Viromed.)
Dr. Weimer of Columbia University has received consulting fees from Roche.)
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.
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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