S3. Autonomic neuropathies — Recent advances: S3.1 Pathogenesis of autoimmune autonomic ganglionopathy

Abstract

Antibodies against nicotinic acetylcholine receptors (AChR) are implicated in myasthenia gravis as well as in autoimmune autonomic ganglionopathy (AAG). AAG typically presents as severe generalized sympathetic and parasympathetic autonomic failure. This disorder is also known as acute pandysautonomia or idiopathic subacute autonomic neuropathy. The most disabling manifestations are orthostatic hypotension (OH) and gastrointestinal dysmotility. An autoimmune pathogenesis was suggested by the subacute onset, frequent association with antecedent viral illness, occasional association with cancer or other autoimmune disorders, and spontaneous recovery in some patients.