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Symptoms of Autoimmune autonomic ganglionopathy
What are the clinical features of Autoimmune autonomic ganglionopathy
Autoimmune autonomic ganglionopathy (formerly known as acute pandysautonomia or idiopathic subacute autonomic neuropathy) is an acute and acquired immune-mediated involvement of both sympathetic and parasympathetic components of the autonomic nervous system, with relative or complete sparing of the somatic nerve fibers.
Antibodies against ganglionic nicotinic acetylcholine receptors (AchR) are found in about 50% of patients.
The typical patient has symptoms related to sympathetic failure (orthostatic hypotension, anhidrosis) or parasympathetic failure (reduced lacrimation and salivation, disturbances of the GI motility [such as ileus, diarrhea, and constipation], bladder atony, impotence, fixed heart rate, and fixed pupils).
The progression of symptoms is rapid in the majority of cases, while some patients have a more insidious onset of disease.
Treatment of AAG primarily consists of supportive care for orthostatic hypotension and bowel and bladder symptoms.
A number of studies have supported the use of intravenous immunoglobulin (IVIG), plasma exchange, and immunomodulatory medications such as prednisone, azathioprine, and rituximab, alone or in combination.
Sources
Mukerji S, Aloka F, Farooq MU, et al.: Cardiovascular complications of the Guillain-Barré syndrome. Am J Cardiol 15:1452, 2009.
