Autoimmune Autonomic Ganglionopathy

Subacute autonomic neuropathies can be further divided into dysautonomia associated with sensory and motor neuropathy, dysautonomia associated with malignancy or idiopathic autoimmune autonomic ganglionopathy (AAG). The most convincing evidence of an autoimmune pathogenesis for AAG is the demonstration of high titers of ganglionic nicotinic acetylcholine receptor (AChR) antibodies in the serum of about 50% of patients. The diagnosis of idiopathic AAG is suspected in cases of acquired autonomic failure without somatic neuropathy when toxic or paraneoplastic causes have been excluded. Patients with paraneoplastic autonomic neuropathy may be clinically indistinguishable from idiopathic AAG until a cancer, usually small-cell carcinoma of the lung, is detected. The original case of acute pandysautonomia was remarkable for highly selective autonomic involvement and complete recovery. Subsequent case series have documented that most patients have a distinct clinical course with monophasic worsening followed by stabilization or remission without recurrences. Treatment for AAG has largely been symptomatic. The convincing evidence that AAG is an antibody-mediated channelopathy supports the use of immunomodulatory therapy for AAG.