Have you watched Snow White and the seven dwarves? The movie revolves around a lovely young princess who is rescued by seven dwarves dwelling in the wilderness. One of the dwarves in the Disney version, “The Sleepy Dwarf” has droopy eyelids not because he is sluggish or drowsy but due to the fact that he is loosely based on Walt Disney’s friend who had Myasthenia Gravis. Myasthenia Gravis is a rare autoimmune disorder that affects nearly all muscles of the human body. In Latin and Greek, it means ‘Grave Muscular Weakness.’ The more you use a muscle, the weaker it becomes. Also, stress and many other factors aggravate the condition. The disease has a reputation for being misunderstood or not understood at all. So we will take a moment to discuss the causes and effects of this paralyzing, deteriorating illness.
What Causes Myasthenia Gravis?
Imagine waking up in the morning and not having the energy to get out of bed. Visualize that you feel drained without knowing why. Picture having to suffer surprising episodes of tingling and pain in your muscles. Imagine not being able to comb your hair or climb up a flight of stairs or laugh loudly. If the picture in your mind scares you then you can fathom the abysmal fear that a myasthenia patient lives in. And to think that the sole cause of all this trouble is an overactive immune system – well, so often it is our own wellwishers that screw us up.
Autoantibodies Attack ACh Receptors
Myasthenia Gravis occurs because your immune system goes into the over-efficient super-man mode. It starts attacking your own body cells. In this manner, it releases antibodies that inhibit, block or attack acetylcholine receptors on the postsynaptic membrane. Thus, the acetylcholine after exiting the nerve ending fails to attach itself to the muscle membrane. Hence the voluntary muscles of the body become weakened and don’t respond to a mental command. So if you are chewing bubble gum and aim to direct the jaw muscles to contract and relax, they might just disappoint you. Why? Simply because the nerve impulse instructing them to perform the action of chewing wasn’t transmitted to the jaw muscles.
Autoantibodies Inhibit Tyrosine Kinase
In addition to stopping the acetylcholine from attaching to the receptor site, some cells of the immune system inhibit the function of tyrosine kinase, a protein that acts as a receptor in muscles. Actually, the antibodies released by the immune system retard the functional capability of tyrosine kinase. This functional defect is directly linked to the development of MG in some patients.
Role Of Thymus Gland
The thymus gland is a component of the immune system, located just beneath your breastbone.
- It is small in healthy adults but large and highly functional in infants. Similarly, it is abnormally large in patients with Myasthenia gravis. It secretes high quantities of antibodies that destroy receptor sites for acetylcholine.
- Not only does it produce antibodies but it also forms cancerous masses called thymomas. Luckily, a thymoma is usually benign and restricted to its point of origin. Doctors often suggest a thymectomy (surgical removal of thymus gland) to reduce the secretion of antibodies.
Development Of Antibody-Negative Myasthenia Gravis
Some people have myasthenia that is not caused by the production of antibodies against muscle-specific tyrosine kinase receptor (MuSK) or acetylcholine(AChR). But the antibodies secreted damage a different kind of protein called lipoprotein-related protein 4 (LRP4). This protein plays a role in maintaining neuromuscular transmission. A research conducted at Georgia Health Sciences University found that Anti-LRP4 antibodies were detected in 9.2% of the patients with negative myasthenia gravis. The researchers concluded that LRP4 is a potential autoantigen in myasthenia patients and the anti-LRP4 autoantibody acts against this protein through various immunopathogenic pathways.
Signs And Symptoms
Life can be bitter and intolerable for a myasthenia patient. These patients experience muscle weakness and fatigue intermittently. A general indicator of MG is general weakness of voluntary muscles. However, we will enumerate the symptoms of Myasthenia gravis here:
- Breathing difficulty
- Limb weakness
- Weakness and fatigue of arm and leg muscles
- Trouble holding up your head sometimes
- Trouble chewing and swallowing
- Pain in the neck and jaws
- Slurred speech and hoarse voice
- Double vision and drooping eyelids
Basically, all of these symptoms don’t present in every patient. The way the disease manifests varies from patient to patient. Also, the severity and the occurrence of different symptoms fluctuate over time. The condition may worsen if left untreated. It is actually sad that muscles deteriorate due to excessive use. The weakness appears in the middle of doing actions. For instance, you may be dismounting a horse and your leg is paralyzed in midair. Imagine having to use crutches for a few days, then go back to normal walking and then switching to crutches again. Life can be unpredictable and full of bad surprises.
Must Read: Myasthenia Gravis: Types Causes And Medication
Diagnosis And Treatment
It is often hard to diagnose myasthenia gravis. Because it is a rare disease and there are multiple diagnoses for muscle weakness, clinicians mistake MG for something else like small fiber neuropathy or multiple sclerosis. Thus patients endure undue suffering before actually being diagnosed.
To identify MG, the physician must:
- Test for muscle weakness in the limbs, eyes, face, and neck
- Examine all the reflexes controlled by reflex arc
- Check for muscle tone
- Verify the loss of sensation and possible tingling in various areas
- Check for visual impairment
Tests
The different tests that can be used to ensure that a patient has MG are:
- Blood Tests to inspect if there is an abnormal release of antibodies
- Tensilon test (Tensilon is a drug that is administered intravenously and then the patient performs various motor functions like running, throwing and picking up a ball e.t.c in front of a doctor.
- Imaging of the chest to check for tumors in the thymus gland
- Repetitive nerve stimulation test to examine nerve damage
Cure Of Myasthenia Gravis
Unfortunately, myasthenia gravis is incurable. But you can take steps to improve and better control your immune system.
Medications
Neurologists prescribe medicines that help muscle activity to improve. Some of these medications are:
- Neostigmine
- Prednisone
- Mycophenolate
- Gravitor/Myestin
- Mestinon
Other corticosteroids and immunosuppressants can also be used that improve the nerve-muscle connection. The immunosuppressants decrease the number of antibodies attacking the acetylcholine. Some of these drugs like neostigmine allow larger than normal amounts of acetylcholine to accumulate in the synaptic space. Within minutes, some of these patients can begin to function almost normally, until a new dose of neostigmine is required a few hours later.
Thymectomy
A thymectomy refers to the surgical removal of the thymus gland. According to the Myasthenia Gravis Foundation of America, 10 to 15 percent of myasthenia patients have a tumor in their thymus gland. As a result, patients experience less muscular fatigue after removing the gland. As these tumors have the potential to be malignant, that is another reason for their removal. Another advantage of getting rid of the gland is a diminished synthesis of autoantibodies.
IVIG
IVIG, which stands for intravenous immune globulin, is donated by healthy individuals and given to myasthenia patients. It helps ameliorate the disease reducing antibody production. The exact mechanism of its action is unknown but it is a successful treatment method.
Plasmapheresis
Plasmapheresis or simply plasma exchange is a clinical technique that extracts harmful antibodies from the blood. The patient can then control his movements voluntarily. The drawback of this method is that it is short-term. As soon as the immune system synthesizes new antibodies, they appear in the blood and myasthenia reoccurs.
Lifestyle Changes
There are a few steps that you can take within the bounds of your home to alleviate myasthenia gravis.
Avoid Stress At All Costs
Stress and anxiety change how the body works. Worry and stress retard nerve stimulation and proper transmission. As a result, you feel drained physically, your muscles tense up and myasthenia presents itself in all its might. Most patients learn very quickly that emotional excitement causes their condition to exacerbate. So doctors suggest patients to not worry at all. But stress is an essential part of life. Think about the positive excitement you feel before doing something new for your personal growth. That is a form of good stress. Conclusively, stress can’t be avoided. The goal should not be to rid yourself of stress but to better cope with it.
Wear an eyepatch if necessary
Some MG patients experience double vision. So they should consult their doctors if it is alright to wear an eyepatch. The eyepatch may improve their vision and focus the image on a single point.
Be grateful and invest in your life
Realise that it is okay to feel scared, depressed, anxious and angry at times. All of us do. Focus on what you have and what you can do with that. You don’t have to like myasthenia to be able to live a healthy life. But you have to accept that you have a few limitations. So invest in your self and utilize your resources to live the best life you possibly can. Also, understand that life is a music composition of fabulous highs and unexpected lows. Take it as it comes to you. All you can do is give it your best shot.
“The deeper that sorrow carves into your being,
The more joy you can contain.”
~Khalil Gibran
Involve Your Family In Your Treatment
If you feel that you are contributing less to your household or that your family doesn’t understand MG, then you need to take them to your checkups. They will meet your doctor and understand how Myasthenia limits your lifestyle choices. They will encourage you to keep smiling and hopefully help you with everyday chores.
Take Plenty Of Rest
The more a myasthenia patient works his muscles, the weaker they become. This is because a repeated stimulus to the muscles is interrupted by the autoantibodies. So put your feet up and take a nap.
Complications Of Myasthenia Gravis
Myasthenia gravis can cause many hurdles along the way. For example, the drugs used to treat MG can lead to excessive weight gain.
Moreover, MG if left untreated can transform into other more fatal autoimmune diseases such as Lupus erythematosus and rheumatoid arthritis.
Myasthenia Crisis
One of the most fatal myasthenia gravis complications is a myasthenia crisis. It is characterized by trouble in breathing properly. So if you face extreme breathing troubles, call 911 or visit the emergency department at your local hospital.
Future Of Myasthenia Gravis
It is true and sad that such a subtle and evil disease is untreatable. But if we, as a community, try to understand it better and help those suffering from it, then and only then can we hope for a future free from this disorder. It wreaks havoc in the patient’s practical life, draining his energy and breaking his strength. Clinical trials for the treatment of MG by hematopoietic stem cell transplant are underway. Other medications are also being processed in pharmaceutical companies and look promising for the future of MG patients.
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