Autoimmune Autonomic Ganglionopathy

Autoimmune autonomic ganglionopathy is an idiopathic acquired disorder of the autonomic nervous system associated with antibodies to the ganglionic nicotinic acetylcholine receptor found in sympathetic, parasympathetic and enteric ganglia. Symptoms and signs reflect diffuse impairment of autonomic functions. Prominent features are gastrointestinal dysmotility, orthostatic hypotension, and tonic pupils. Typical cases have a subacute onset (less than 3 months to maximum symptoms), are monophasic, and may show partial improvement over the course of several months. Other cases have a slowly progressive course which can resemble degenerative forms of autonomic failure. Treatment for milder cases is supportive care for symptom management. Anecdotally, plasma exchange, intravenous immunoglobulin, corticosteroids or immunosuppression have been used successfully to treat more severe cases. Autoimmune autonomic ganglionopathy represents one of a small group of autoimmune neuromuscular disorders that are caused by antibodies against ion channels.