Autoimmune autonomic disorders

Andrew B McKeon, Eduardo E. Benarroch

Research output: Chapter in Book/Report/Conference proceedingConference contribution

13 Scopus citations

Abstract

Autoimmune autonomic disorders occur because of an immune response directed against sympathetic, parasympathetic, and enteric ganglia, autonomic nerves, or central autonomic pathways. In general, peripheral autoimmune disorders manifest with either generalized or restricted autonomic failure, whereas central autoimmune disorders manifest primarily with autonomic hyperactivity. Some autonomic disorders are generalized, and others are limited in their anatomic extent, e.g., isolated gastrointestinal dysmotility. Historically, these disorders were poorly recognized, and thought to be neurodegenerative. Over the last 20 years a number of autoantibody biomarkers have been discovered that have enabled the identification of certain patients as having an autoimmune basis for either autonomic failure or hyperactivity. Peripheral autoimmune autonomic disorders include autoimmune autonomic ganglionopathy (AAG), paraneoplastic autonomic neuropathy, and acute autonomic and sensory neuropathy. AAG manifests with acute or subacute onset of generalized or selective autonomic failure. Antibody targeting the α3 subunit of the ganglionic-type nicotinic acetylcholine receptor (α3gAChR) is detected in approximately 50% of cases of AAG. Some other disorders are characterized immunologically by paraneoplastic antibodies with a high positive predictive value for cancer, such as antineuronal nuclear antibody, type 1 (ANNA-1: anti-Hu); others still are seronegative. Recognition of an autoimmune basis for autonomic disorders is important, as their manifestations are disabling, may reflect an underlying neoplasm, and have the potential to improve with a combination of symptomatic and immune therapies.

Original languageEnglish (US)
Title of host publicationAutoimmune Neurology, 2016
PublisherElsevier
Pages405-416
Number of pages12
Volume133
ISBN (Print)9780444634320
DOIs
StatePublished - 2016

Publication series

NameHandbook of Clinical Neurology
Volume133
ISSN (Print)00729752
ISSN (Electronic)22124152

Keywords

  • Autoimmune
  • Dysautonomia
  • Ganglionopathy
  • Paraneoplastic

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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