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Neuromuscular and nervous system

Autoimmune autonomic ganglionopathy

Autoimmune autonomic ganglionopathy is a rare condition in which the immune system is thought to target the ganglionic acetylcholine receptor, a protein that helps relay signals at the autonomic ganglia, the relay stations for the nerve pathways that run automatic body functions like blood pressure, digestion, and pupil response. Antibodies against this receptor disrupt synaptic transmission in those ganglia and are found in roughly half of patients, with higher antibody levels generally associated with more severe symptoms. The result is a fairly rapid, often subacute onset of widespread autonomic failure that is usually monophasic, meaning it happens once, with some patients seeing partial improvement over months.

The condition is considered rare enough that solid population-wide prevalence figures are not well established. A case series reported a median age at diagnosis around 45.

Common signs

Severe lightheadedness or fainting on standing from a drop in blood pressure (orthostatic hypotension), dry mouth and dry eyes, constipation and other gut sluggishness, bladder problems, and pupils that react poorly to light.

This is a plain-language summary for orientation, not a diagnosis. Mechanisms are described as current understanding, not settled fact. Work with your own clinician for your care.

Questions people ask

What causes autoimmune autonomic ganglionopathy?+
The immune system is thought to target the ganglionic acetylcholine receptor, a protein that helps relay signals at the autonomic ganglia, the relay stations for the nerve pathways that run automatic body functions like blood pressure, digestion, and pupil response. These antibodies disrupt synaptic transmission in those ganglia and are found in roughly half of patients, and higher antibody levels are generally associated with more severe symptoms.
What are the symptoms of autoimmune autonomic ganglionopathy?+
Symptoms include severe lightheadedness or fainting on standing from a drop in blood pressure (orthostatic hypotension), dry mouth and dry eyes, constipation and other gut sluggishness, bladder problems, and pupils that react poorly to light. Onset is fairly rapid and often subacute, and the condition is usually monophasic, meaning it happens once, with some patients seeing partial improvement over months.
Is autoimmune autonomic ganglionopathy the same as other conditions that cause a racing heart or fainting on standing?+
This page describes autoimmune autonomic ganglionopathy specifically and does not compare it to other autonomic conditions, so it doesn't provide that distinction. If you want to understand how your symptoms fit a specific diagnosis, a neurologist is the right person to sort that out.
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Daniela Hess, MSEd, is a co-founder of Great Energy and an Autoimmune Educator and Functional Wellness Consultant who lives and thrives with Hashimoto’s. She is not a licensed medical provider.

Meet Your Guide, Daniela Hess, the Founder and Creator of the Autoimmune Recovery MethodThe 3 factors behind every Autoimmune conditionThe Autoimmune Diseases: What Autoimmunity Really Is, and Why It Lands Where It Does

AIP recipes to try

A place to start in the kitchen while you work on this with your own clinician: