Autoimmune autonomic ganglionopathy

Autoimmune autonomic ganglionopathy
Other namesAutoimmune autonomic neuropathy, Acute pandysautonomia
Functions of the autonomic nervous system.
SpecialtyNeurology 
SymptomsGastrointestinal dysmotility, orthostatic hypotension, and tonic pupils.[1]
ComplicationsWeight loss.
Diagnostic methodClinical criteria and serum ganglionic neuronal nicotinic AChR antibodies.
Differential diagnosisParaneoplastic syndrome, Guillain-Barré syndrome, diabetes, amyloidosis, Sjogren's syndrome, and Morvan syndrome.

Autoimmune autonomic ganglionopathy is a type of immune-mediated autonomic failure that is associated with antibodies against the ganglionic nicotinic acetylcholine receptor present in sympathetic, parasympathetic, and enteric ganglia. Typical symptoms include gastrointestinal dysmotility, orthostatic hypotension, and tonic pupils.[1] Many cases have a sudden onset, but others worsen over time, resembling degenerative forms of autonomic dysfunction. For milder cases, supportive treatment is used to manage symptoms.[2] Plasma exchange, intravenous immunoglobulin, corticosteroids, or immunosuppression have been used successfully to treat more severe cases.[1]

  1. ^ a b c Winston, Nicole; Vernino, Steven (2009). "Autoimmune Autonomic Ganglionopathy". Frontiers of Neurology and Neuroscience. Vol. 26. Basel: KARGER. pp. 85–93. doi:10.1159/000212370. ISBN 978-3-8055-9141-6. ISSN 1660-4431. PMID 19349706.
  2. ^ Wang, Z.; Low, P. A.; Jordan, J.; Freeman, R.; Gibbons, C. H.; Schroeder, C.; Sandroni, P.; Vernino, S. (May 29, 2007). "Autoimmune autonomic ganglionopathy: IgG effects on ganglionic acetylcholine receptor current". Neurology. 68 (22). Ovid Technologies (Wolters Kluwer Health): 1917–1921. doi:10.1212/01.wnl.0000263185.30294.61. ISSN 0028-3878. PMC 2615674. PMID 17536048.