Autoimmune Autonomic Ganglionopathy

Steven Vernino, Phillip Anson Low

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Subacute autonomic neuropathies can be further divided into dysautonomia associated with sensory and motor neuropathy, dysautonomia associated with malignancy or idiopathic autoimmune autonomic ganglionopathy (AAG). The most convincing evidence of an autoimmune pathogenesis for AAG is the demonstration of high titers of ganglionic nicotinic acetylcholine receptor (AChR) antibodies in the serum of about 50% of patients. The diagnosis of idiopathic AAG is suspected in cases of acquired autonomic failure without somatic neuropathy when toxic or paraneoplastic causes have been excluded. Patients with paraneoplastic autonomic neuropathy may be clinically indistinguishable from idiopathic AAG until a cancer, usually small-cell carcinoma of the lung, is detected. The original case of acute pandysautonomia was remarkable for highly selective autonomic involvement and complete recovery. Subsequent case series have documented that most patients have a distinct clinical course with monophasic worsening followed by stabilization or remission without recurrences. Treatment for AAG has largely been symptomatic. The convincing evidence that AAG is an antibody-mediated channelopathy supports the use of immunomodulatory therapy for AAG.

LanguageEnglish (US)
Title of host publicationPrimer on the Autonomic Nervous System
PublisherElsevier Inc.
Pages489-492
Number of pages4
ISBN (Print)9780123865250
DOIs
StatePublished - 2012

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Primary Dysautonomias
Paraneoplastic Polyneuropathy
Channelopathies
Immunomodulation
Antibodies
Poisons
Small Cell Lung Carcinoma
Nicotinic Receptors
Neoplasms
Recurrence
Serum
Therapeutics

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Vernino, S., & Low, P. A. (2012). Autoimmune Autonomic Ganglionopathy. In Primer on the Autonomic Nervous System (pp. 489-492). Elsevier Inc.. https://doi.org/10.1016/B978-0-12-386525-0.00100-1

Autoimmune Autonomic Ganglionopathy. / Vernino, Steven; Low, Phillip Anson.

Primer on the Autonomic Nervous System. Elsevier Inc., 2012. p. 489-492.

Research output: Chapter in Book/Report/Conference proceedingChapter

Vernino, S & Low, PA 2012, Autoimmune Autonomic Ganglionopathy. in Primer on the Autonomic Nervous System. Elsevier Inc., pp. 489-492. https://doi.org/10.1016/B978-0-12-386525-0.00100-1
Vernino S, Low PA. Autoimmune Autonomic Ganglionopathy. In Primer on the Autonomic Nervous System. Elsevier Inc. 2012. p. 489-492 https://doi.org/10.1016/B978-0-12-386525-0.00100-1
Vernino, Steven ; Low, Phillip Anson. / Autoimmune Autonomic Ganglionopathy. Primer on the Autonomic Nervous System. Elsevier Inc., 2012. pp. 489-492
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