Autoimmune autonomic ganglionopathy: an update on diagnosis and treatment

Expert Rev Neurother. 2018 Dec;18(12):953-965. doi: 10.1080/14737175.2018.1540304. Epub 2018 Nov 1.

Abstract

Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to autonomic failure. The disorder is associated with autoantibodies to the ganglionic nicotinic acetylcholine receptor (gAChR). We subsequently reported that AAG is associated with an overrepresentation of psychiatric symptoms, sensory disturbance, autoimmune diseases, and endocrine disorders. Area covered: The aim of this review was to describe AAG and highlight its pivotal pathophysiological aspects, clinical features, laboratory examinations, and therapeutic options. Expert commentary: AAG is a complex neuroimmunological disease, these days considered as an autonomic failure with extra-autonomic manifestations (and various limited forms). Further comprehension of the pathophysiology of this disease is required, especially the mechanisms of the extra-autonomic manifestations should be elucidated. There is the possibility that the co-presence of antibodies that were directed against the other subunits in both the central and peripheral nAChRs in the serum of the AAG patients. Some patients improve with immunotherapies such as IVIg and/or corticosteroid and/or plasma exchange. 123I-MIBG myocardial scintigraphy may be a useful tool to monitor the therapeutic effects of immunotherapies.

Keywords: Ganglionic acetylcholine receptor; autoantibodies; autoimmune autonomic ganglionopathy; extra-autonomic manifestations; immunotherapy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • 3-Iodobenzylguanidine
  • Adrenal Cortex Hormones / therapeutic use
  • Autoantibodies / blood
  • Autoimmune Diseases of the Nervous System / diagnosis*
  • Autoimmune Diseases of the Nervous System / immunology
  • Autoimmune Diseases of the Nervous System / therapy*
  • Ganglia, Autonomic / immunology*
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Myocardial Perfusion Imaging
  • Plasma Exchange
  • Radiopharmaceuticals
  • Receptors, Nicotinic / immunology

Substances

  • Adrenal Cortex Hormones
  • Autoantibodies
  • Immunoglobulins, Intravenous
  • Radiopharmaceuticals
  • Receptors, Nicotinic
  • 3-Iodobenzylguanidine