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Autoimmune autonomic ganglionopathy (AAG) is a rare autoimmune disorder with antibodies against ganglionic nicotinic acetylcholine receptor (α3-nAChR) of the autonomic ganglia¹. Patients may experience symptoms of acute to subacute, generalized sympathetic, parasympathetic and enteric autonomic failure. Antibodies are detected in about 50% of patients and levels correlate with the severity of dysautonomia. An understanding of the underlying pathophysiology is imperative for timely diagnosis and management, including the appropriate use of immunosuppressive therapy in conjunction with plasmapheresis, supportive care and a search for malignancy. Patients may be seronegative for α3-nAChR antibodies but may still benefit from immunotherapy³. The clinical course of AAG is variable. Spontaneous improvement occurs in about one-third of patients, but recovery is typically incomplete.

The ANS affects blood flows, digestion, the immune system, heart rate, sleep, etc. When the body needs to respond to a stressor, the fight or flight (sympathetic nervous system (SNS)) is activated. When the stressor is gone, the “rest and digest” (parasympathetic nervous system (PNS)) returns the body to normal functioning. In ME/CFS, POTS and FM, the fight or flight system has become unusually activated.

There was a talk on autoimmune autonomic gangliopathy (AAG) given by Steven Vernino at the recent Dysautonomia Conference.

Autoimmune autonomic gangliopathy (AAG) is interesting because it demonstrates how the immune system can take a two-by-four to the autonomic nervous system. Given the many people with ME/CFS, FM and POTS whose diseases were triggered by an infection; i.e. an immune response – that’s an intriguing fact.

Steve Vernino related how one 50 year old woman’s AAG began with a cold! Four days later, she was admitted to hospital with severe nausea, abdominal pain, tingling, blurry vision, and dizziness. Her blood pressure was doing weird things: lying down, her blood pressure was high normal, but standing up, it dropped to 80/56.

Since the ANS regulates blood pressure, those strange blood pressure readings suggested that her ANS wasn’t functioning properly. Other signs – dry mouth, enlarged pupils, and lack of sweating – also pointed to problems with the ANS. Her sensory and motor nerves – which run alongside ANS nerves – on the other hand, were normal.

What had happened? Autoimmune autonomic ganglionopathy (AAG) is a rare disease, but researchers ultimately uncovered what was going on. The ANS produces two kinds of nerve “ganglia” – areas where the nerves collect together. The parasympathetic nerve ganglia are found near the organs, while the sympathetic ganglia are found close to the spinal cord. Acetylcholine is used to open up a channel between one neuron and another – and activate the nerves.

Studies uncovered the presence of antibodies in AAG patients which attack the anti-nicotinic acetylcholine receptors (AChR) that are responsible for opening up the channel between two autonomic nerves. With the channel blocked, the signal cannot pass and the ANS cannot work. That’s why her blood pressure dropped severely when standing up. It’s why she had so many gut problems.

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