Autonomic Neuropathies

Valeria Iodice, MD, PhD; Paola Sandroni, MD, PhD, FAAN Peripheral Nervous System Disorders p. 1373-1397 October 2014, Vol.20, No.5 doi: 10.1212/01.CON.0000455875.76179.b1
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Purpose of Review: This article focuses on the most prevalent forms of autonomic neuropathies, but also discusses conditions such as focal and dysfunctional syndromes (altered autonomic function in the absence of structural lesions). The goal of this review is to allow the reader to promptly recognize these disorders, identify potentially reversible or treatable causes, and implement the appropriate treatment as well as supportive care.

Recent Findings: Secondary forms of autonomic neuropathies (eg, diabetes mellitus, amyloidosis) are much more common than primary forms, of which autoimmune ganglioneuropathies represent a major component. However, the spectrum of the latter is continuously evolving and has diagnostic and therapeutic implications. Testing modalities such as autonomic testing, serum autoimmune antibody testing, and skin biopsies are becoming more widely available.

Summary: Autonomic neuropathies are relatively common conditions, and, because of the prognostic implications as well as impact on patient quality of life, they should be promptly recognized and treated aggressively. Testing is critical as other conditions may mimic autonomic neuropathies. Treatment is symptomatic in many cases, but specific therapies are also available in selected autonomic neuropathies.

Address correspondence to Dr Valeria Iodice, University College London, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK, WC1N 3BG, [email protected].

Relationship Disclosure: Drs Iodice and Sandronireport no disclosures.

Unlabeled Use of Products/Investigational Use Disclosure: Drs Iodice and Sandroni discuss the unlabeled use of ephedrine, octreotide, acarbose, voglibose, erythropoietin, desmopressin, yohimbine, pyridostigmine, and dihydroergotamine for the treatment of orthostatic hypotension.

© 2014 American Academy of Neurology