Do you sometimes suddenly feel dizzy? Or do you feel like there's something strange in your stomach, your mouth is dry, or you can't control your breathing? Even though these may seem like normal things, sometimes there can be a more complicated story behind them. That's one of those rare, but worth knowing, conditions that we're going to talk about today.
What is this ``Autoimmune Autonomic Ganglionopathy - AAG''? Let's understand it simply, shall we?
Simply put, ``Autoimmune Autonomic Ganglionopathy'' or ``(AAG)'' is a condition in which our own body's defense system, that is, the immune system, mistakenly attacks our own autonomic nervous system. Imagine, this is like what would happen if the army that is supposed to protect our country mistakenly attacked our own important places.
Our body's autonomic nervous system is like our ``automatic system''. That is, it controls many things that happen automatically, without us realizing it. For example:
- Your breathing
- Heart rate (heart rate)
- Blood pressure
- Sweating
- Digestion
Many of these things are not something we think about. They happen automatically. The autonomic nervous system is what helps keep those automatic functions running smoothly. In AAG, this important system is damaged. This is a condition called autonomic neuropathy, or dysautonomia.
Why does our own body do this? What are the reasons?
In fact, even doctors still can't say exactly what the exact cause of this `(AAG)` is. But some facts have been discovered.
- Most of the time, that is , in about three out of every five people who develop ``AAG'', this condition occurs after recovering from another illness, or after an infection. It's like a side effect of that illness.
- Some people can develop a condition called paraneoplastic AAG. This is when some antibodies that our immune system makes in response to a condition like cancer go and attack the autonomic nervous system.
What does this do to our bodies? Let's take a closer look.
Our autonomic nervous system has clusters of nerve cells called autonomic ganglia . These are like train junctions. Various messages are sent and received through these. This exchange of messages is essential for the automatic actions of our body, that is, the actions that we do not control (reflexes), to be controlled.
In AAG, our immune system attacks specific receptors in these ganglia. Receptors are the places where messages are received. So when these receptors are damaged, those nerve messages don't go or get through properly. As a result, things that happen automatically, like controlling blood pressure and sweating, start to go haywire.
Imagine, if a `connection` in your home's `main switch board` becomes loose, the lights won't turn on, the fan won't spin, etc. That's how it is with this.
How common is this condition called `(AAG)`?
This is actually a very rare condition. For example, in the United States, only about 100 people are diagnosed with this disease each year. So it is very rare in Sri Lanka. So don't be afraid when you hear about this, this is not something that happens to many people.
What are the symptoms of `(AAG)`? How do we recognize them?
The symptoms of this condition can vary from person to person. Not everyone will experience the same symptoms. The most common symptoms include:
- Constipation: This means constipation and difficulty going to the toilet.
- Dilated pupils (Adie's pupils): Sometimes only one eye's pupil may be dilated and may be sensitive to light.
- Dry mouth or dry eyes: A feeling as if there is no saliva or tears in the eyes.
- Fainting / Syncope: You may suddenly faint for no apparent reason.
- Neurogenic orthostatic hypotension: A feeling of dizziness and lightheadedness when standing up from a sitting position or getting out of bed. This is caused by a sudden drop in blood pressure.
- Difficulty urinating / Urinary retention: The feeling of not being able to urinate even though you want to urinate.
About two out of every three people with AAG have been found to have high levels of antibodies called ganglionic acetylcholine receptor antibodies (g-AChR antibodies) in their blood. Researchers believe that there is a link between the levels of these g-AChR antibodies and the severity of symptoms.
How does a doctor diagnose this `(AAG)` disease?
If you have symptoms like these, the first thing a doctor will do is ask you about your symptoms and examine you. Then, they may do some special tests to check the functioning of your autonomic nervous system.
Here are some of them:
- Digestive system tests: For example, a ``Gastric emptying`` test. This tests how quickly food leaves your stomach.
- `Quantitative sudomotor axon reflex test (QSART):` This measures how your sweat glands are working.
- `Thermoregulatory sweat test`: This test is performed to find out whether the body's sweating has decreased or increased, and what the cause is.
- `Tilt table test`: In this test, you are made to lie down on a special bed and the bed is slowly rotated vertically, observing how your heart rate and blood pressure change.
- Urinalysis: This can give you an idea of how well your bladder is functioning.
In addition, a blood test may be done to check the level of the aforementioned `(g-AChR antibodies)` . If the level of this antibody is high, it is further evidence that it may be `(AAG)`. However, this blood test is different from the `AChR antibody test`, which is used to check for another neurological disease called `(Myasthenia Gravis).`
Is there a treatment for this? What can be done?
Because AAG is such a rare condition, there is no standard, "this is the treatment" yet. Most of the time , the focus is on symptom management.
Doctors create a treatment plan by combining various treatment methods. Some of them include:
- Plasma exchange / plasmapheresis: This involves taking some of your blood, removing harmful substances like antibodies from the plasma, and then giving the purified blood back to your body.
- Intravenous immunoglobulin (IVIG) therapy: This involves giving your body immunoglobulin (a part of plasma that fights disease) taken from healthy people. This helps to calm the immune system.
- Intravenous corticosteroids: High doses of anti-inflammatory steroids, such as methylprednisolone (Solu-Medrol®) or hydrocortisone (Solu-Cortef®), are given by injection.
- Immunosuppressive medications: These work by reducing the overactivity of the immune system. For example, medications like rituximab (Rituxan®).
Is there a way to prevent this `(AAG)` situation?
There's really no guaranteed way to prevent this, as it's very rare. However, some conditions, like cancer, can slightly increase your risk of developing it. If you've had cancer, here are some things you can do to take care of your overall health:
- Regular exercise: especially aerobic exercise.
- If you have `(Dysautonomia)`, eating a diet high in salt and fluids can help control your blood pressure. (This is something you should discuss with your doctor.)
- Managing stress: Use healthy methods like exercising, talking to a friend, and writing in a journal.
- Get good sleep: Sleep at least 7-8 hours a day.
- Wearing compression garments: These help improve blood circulation and reduce swelling (especially in the legs).
Can this `(AAG)` situation be completely cured? What will happen in the future?
Unfortunately, AAG is not curable. However, many people can manage their symptoms well over the long term. Surprisingly, about one in three people with AAG will gradually get better without any treatment. However, most people will have to deal with some symptoms throughout their lives.
What else do you want to ask the doctor?
If you suspect you have a condition like this, it's very important to see a good doctor and ask about the following:
- What is the most likely cause of my symptoms?
- What tests should I do to find out exactly if I have `(AAG)`?
- What are the treatment options for `(AAG)`?
- What is the likelihood that the symptoms of `(AAG)` will subside even without treatment?
- What lifestyle changes should I make to improve my overall health?
What other immune conditions are associated with autonomic neuropathy?
``(AAG)`` is special because it is, on the one hand, a disease related to the immune system, and on the other, an autoimmune disease of the nervous system.
Additionally, there are some other immune system-related diseases that increase the risk of developing ``dysautonomia'' (disorders of the autonomic nervous system). Some examples are:
- Celiac disease
- `(Guillain-Barré syndrome)` (Guillain-Barré syndrome)
- `(Lupus / systemic lupus erythematosus)` (Lupus disease)
- `(Rheumatoid arthritis)` (Rheumatoid arthritis)
- `(Sjögren's syndrome)` (Sjögren's syndrome)
So you can see, these are situations that can be interconnected.
Finally, things to remember (Take-Home Message)
Okay, so now you know that `(Autoimmune Autonomic Ganglionopathy - AAG)` is a rare disease in which our own defense system attacks our autonomic nervous system. Symptoms can include sudden loss of consciousness, a drop in `pressure` when standing, and stomach problems.
Don't be alarmed when you hear this. This is very rare. Also, immunosuppressant drugs and `plasmapheresis` are used as treatments for this. Although it cannot be cured completely, most people can control the symptoms well and live a normal life.
The most important thing is to see a qualified doctor as soon as possible if you have these symptoms. If you recognize them early, you can start treatment and reduce the impact on your life. You are not alone, and there are doctors who can help.
` Autoimmune Aneurysmal Ganglionopathy, AAG, Autoimmune Nervous System, Immune System, Neurological Disease, Symptoms, Treatment
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