Autoimmune autonomic ganglionopathy

Clinic

• AAG is a rare form of dysautonomia in which the immune system produces
  • Ganglionic anti-nicotinic acetylcholine receptor (AChR) antibodies,
  • Inhibiting ganglionic AChR currents and
  • Impairing transmission in autonomic ganglia

• Symptoms onset can be acute, subacute or gradual

Signs / Symptoms

Although symptoms of AAG can vary from patient to patient, symptoms are dysautonomia. Hallmarks include:

• Gastrointestinal dysmotility, including lack of appetite, nausea, constipation, diarrhea
• Anhidrosis, often preceded by excessive sweating
• Neurogenic bladder
• Small fiber peripheral neuropathy
• Severe Orthostatic hypotension
• Pupillary dysfunction
• Syncope
• Sicca syndrome / Xerostomia

Causes

• The cause is generally either paraneoplastic syndrome or idiopathic. In idiopathic AAG, the body's own immune system targets a receptor in the autonomic ganglia, which is part of a peripheral nerve fiber. If the AAG is paraneoplastic, they have a form of cancer, and their immune system has produced paraneoplastic antibodies in response to the cancer.

Related Disease

• Multiple system atrophy: History or physical examination of cerebellar, striatal, pyramidal, and extrapyramidal dysfunction