Disorders In Depth
Autoimmune Autonomic Ganglionopathy
Introduction
Autoimmune autonomic ganglionopathy (AAG) is a rare disorder characterized by the presence of autonomic failure in association with specific antibodies directed against the α3 subunit of the neuronal nicotinic acetylcholine receptor (AChR) of the autonomic ganglia. The cause is unknown.
Symptoms
- Severe orthostatic hypotension (lasting weeks to years)
- Syncope
- Constipation
- Urinary retention
- Fixed and dilated pupils
- Dry mouth and eyes
Treatment
- For mild cases, treatment is centered around symptom management.
- Many patients remain incapacitated and disease modifying therapy may help.
- There is no established therapeutic regimen.
- A therapeutic trial in autoimmune autonomic ganglionopathy is being conducted by the Autonomic Disorders Consortium
