Autoimmune Autonomic Ganglionopathy - Causes, Symptoms, Diagnosis, and Treatment

What Is Autoimmune Autonomic Ganglionopathy?

Autoimmune autonomic ganglionopathy (AAG) is an autoimmune condition that affects the nervous system. The body’s immune system perceives threats from the nerve cells and begins to attack them. In this condition, the autonomic nervous system is affected, which forms a part of the peripheral nervous system. The autonomic nervous system controls breathing, heart rate, and blood pressure. AAG can also be regarded as dysautonomia-autonomic nervous system disorders.

What Happens in Autoimmune Autonomic Ganglionopathy?

In autoimmune autonomic ganglionopathy, a specific receptor of the nervous system is damaged by the immune cells. In AAG, the autonomic ganglia are affected. The ganglia are nerve cells that pass and process electrical impulses. The electrical impulses generate signals that control various activities of the body. In the case of the autonomic nervous system, these impulses control breathing, heart rate, and blood pressure. When these receptors are damaged due to immune activation, the other functions are also affected. This will affect the regulation of blood pressure, breathing, and heart rate regulation.

What Are the Causes of Autonomous Autonomic Ganglionopathy?

The exact cause for the occurrence is unknown. However, the following can be a possible contributing factor to the occurrence of AAG. The possible causes include the following:

1. Infection: The occurrence of AAG is more commonly seen after an infection. This condition develops a few months or weeks after the infection is cured. AAG is seen in three out of five cases as a consequence of infection

2. Paraneoplastic AAG: Paraneoplastic AAG is seen in cancer patients. The body’s immune system makes certain antibodies to respond to cancer cells. These immune cells attack the autonomic nervous system, which leads to conditions like AAG. Patients suffering from conditions like cancer are more likely to develop AAG.

3. Inherited conditions: Sometimes, conditions like AAG can be inherited. It can be passed on from the parents to the children on familial inheritance patterns.

4. Emotional Stress: Many autoimmune conditions occur due to acute exacerbation of emotional stress. Conditions such as GBS (Guillain-Barre syndrome) which affects the autonomic nervous system, occur due to stress.

What Are the Symptoms of Autoimmune Autonomic Ganglionopathy?

The symptoms of AAG vary based on the severity of the condition. However, some of the most common symptoms of AAG include the following:

1. Dizziness: The most common symptoms of AAG include dizziness and fainting. This is experienced as a result of a drop in blood pressure levels.

2. Dilation of the Pupils: The dilation of the pupils can be due to affected ganglia. The pupils tend to remain dilated both in bright light and darkness.

3. Dryness of the Eyes and Mouth: The autonomic nervous system is responsible for saliva and tear production. Hence when this is affected, there can be dryness of the eyes. Oral dryness will also be felt due to a reduction in saliva production.

4. Increase or Decrease in Sweating: Like the tears and saliva, the sweat glands are also controlled by the autonomic nervous system. Therefore in AAG, there can be an increase to regulate sweating. There is an inability to regulate body temperature.

5. Orthostatic Intolerance: Orthostatic intolerance refers to the sudden drop in blood pressure with posture change. This is commonly seen in AAG.

6. Urine Retention: AAG can cause urine retention and bladder problems such as:

• Lacking bladder control.

• Leaking urine.

• Inability to start urinating.

• Inability to sense the fullness of the bladder.

• Inability to empty the bladder.

7. Digestive Issues: When AAG affects the autonomic nerves of the digestive system, the following symptoms can be elucidated:

• Loss of appetite.

• Feeling full in just a little.

• Constipation.

• Diarrhea.

• Bloating.

• Nausea.

• Vomiting.

• Heartburn.

• Difficulty in swallowing.

How to Diagnose Autonomic Autoimmune Ganglionopathy?

The diagnosis of AAG can be made by comparing various parameters such as symptoms and other diagnostics. The various tests that are performed to check for AAG include:

1. Antibodies in Blood: In people with AAG, ganglionic acetylcholine receptor antibodies (g- AChR antibodies) are at higher levels in the blood. These antibodies are responsible for the attack on the ganglia of the nervous system. The higher the levels of antibodies, the more the symptoms.

2. Tilt Table Test: This test checks for the variation in blood pressure and heart rate with changing positions. The patient is made to lie on a table, and the degree of tilt is changed while the heart rate and blood pressure are monitored continuously.

3. Thermoregulatory Test: This test aims at evaluating sweating. It also checks the body’s temperature regulation.

4. Urine Analysis: The urine analysis helps to analyze the bladder's functioning. It analyzes bladder emptying and its other parameters.

5. Quantitative Sudomotor Axon Reflex Test (QSART): This test measures the functioning capacity of the sweat glands. Since the sweat glands are directly controlled by the autonomic nervous system. This test can help in the diagnosis of AAG.

How Is Autoimmune Autonomic Ganglionopathy Treated?

Since AAG is very rare, there is no existing standard treatment protocol for its treatment. The treatment for AAG is a combination of various methodologies, which include:

1. Treatment With Steroids: The most common treatment protocol for any autoimmune condition is treated with steroids. Steroids such as methylprednisolone are given in the intravenous route to manage AAG.

2. Intravenous Immunoglobulin Treatment (IVIG): Immunoglobulins give fighting properties to immune cells. Treating such plasma substances improves the disease-fighting capacity of the body.

3. Immunosuppressants: To fight AAG, immunosuppressants such as Rituximab are given. Immunosuppressants help in reducing the impact of the immune cells on the body.

4. Plasma Exchange: The plasma exchange process is similar to that of dialysis. In plasma exchange, the blood is drawn out of the body, and plasma is purified of harmful substances and returned to the body.

Is AAG Curable?

AAG is not curable. But this can be managed. The symptoms of AAG can be managed when appropriate treatment is given. In many cases, the AAG can resolve independently and never return. However, care should be taken to prevent its recurrence.

Conclusion:

AAG is a condition that affects the autonomic nervous system. It can affect various parameters controlled by the autonomic nervous system, such as heart rate, breathing, and blood pressure. The exact cause of the condition is unknown, but it can occur in cancer patients or after an infection. Once it has affected a person, appropriate treatment should be taken. Also, precautions should be taken to prevent its recurrence.