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No two snowflakes are alike. And no two cases of myasthenia gravis, popularly nicknamed the “snowflake disease,” are alike either. The result of an overactive immune system, myasthenia gravis causes bouts of muscle weakness and fatigue. There’s more than one cause, so symptoms and treatment can vary. Although it takes some trial and error, most people with myasthenia gravis will find treatments that dramatically, if not completely, alleviate the weakness.
What is myasthenia gravis?
Myasthenia gravis is an autoimmune disorder characterized by fluctuating muscle weakness that worsens with exertion and improves with rest. Myasthenia gravis is a rare condition. About two in every 10,000 people live with the condition. Most experience only mild or moderate symptoms.
The weakness concentrates on one or more muscle groups, typically the eyes and eyelids. However, the jaw muscles, throat, upper extremities (thighs and biceps), and, in some cases, the respiratory muscles are affected
Like other autoimmune disorders, the symptoms of myasthenia gravis are caused by an overactive immune system. The body mistakenly produces antibodies that attach to receptors at the neuromuscular junction, the place where nerves pass signals to muscle cells and stimulate muscle contractions. Nerves communicate to muscle cells through a chemical called acetylcholine, but the antibodies attack the receptors that allow these signals to get through. Weakened nerve signals cause the fluctuating weakness typical of myasthenia gravis.
Several types of autoantibodies cause myasthenia gravis, named after the receptor to which they attach: AChR antibodies, MuSK antibodies, or LRP4 antibodies. Symptoms and treatment will vary depending on the type of autoantibody causing the disease.
No cause has been discovered, but the thymus gland is often involved. Around 10%–15% of cases involve tumors of the thymus gland. This organ, located in the upper chest, produces immune cells that generate antibodies. Again, both symptoms and treatment can vary depending on the involvement of the thymus.
Finally, myasthenia gravis is classified based on the age of onset and the extent that it affects the body, either limited to the eyes (ocular myasthenia gravis) or muscle groups elsewhere (generalized myasthenia gravis). Again, symptoms and treatment may vary.
The most serious complication of myasthenia gravis is a myasthenic crisis, a medical emergency that happens when respiratory muscles become too weak to allow for sufficient breathing. Patients will require hospitalization and may need to be put on a mechanical ventilator. Other complications include neonatal myasthenia gravis. Pregnant women with myasthenia gravis can pass antibodies to the fetus, and the baby may be born with myasthenia gravis. It is, however, only a temporary condition and resolves in a few weeks or months.
Myasthenia gravis is highly treatable. Most patients will experience significant if not total remission of symptoms from a variety of medications or procedures. About 10%–15% of patients with generalized myasthenia gravis, however, have severe symptoms that cannot be adequately controlled by medications, a condition called refractory myasthenia gravis.
How is myasthenia gravis diagnosed?
Myasthenia gravis is diagnosed mainly from the symptoms and patient’s history, but testing is required to determine the type and severity of the condition. Testing, final diagnosis, and treatment will primarily be managed by a neurologist.
The characteristic symptom of myasthenia gravis is weakness that comes and goes, gets worse with exertion or repetitive activities, and improves with rest.
Other typical symptoms depend on which muscle groups are affected and include:
- Blurred vision
- Double vision
- Drooping eyelid (ptosis)
- Impaired speech
- Difficulty chewing or swallowing
- Choking
- Expressionless face or drooping head
- Weak upper arms or legs
- Difficulty breathing
Because myasthenia gravis is diagnosed by the type of autoantibodies attacking the neuromuscular junctions, blood tests will be required to identify those antibodies. In patients who do not have myasthenia gravis autoantibodies, other tests may be required:
- Electrophysiological tests such as repetitive nerve stimulation (RNS) or electromyography (EMG) can diagnose delays in nerve interactions with muscles
- Edrophonium test is used when electrophysiologic tests can’t be performed, for instance, when the weakness is limited to the eyes. Edrophonium is a drug that rapidly improves the ability of nerves to communicate with muscles. The neurologist will monitor the patient for improvements in double vision or droopy eyelids
- If none of these tests can be administered, an ice pack test works in much the same way as an edrophonium test. Cold typically improves nerve conduction in myasthenia gravis, so an ice pack is placed over the eye for two to five minutes to see if it improves double vision or drooping eyelid
A CT scan of the chest will be performed if the doctor suspects cancer of the thymus gland (thymoma)
Myasthenia gravis treatment options
Myasthenia gravis is an autoimmune disorder that cannot be cured. The goal of long-term treatment is to achieve a partial or total remission of symptoms with the least amount of unwanted side effects. This is achieved by drugs that increase the ability of nerves to pass signals to the muscles, drugs that suppress the immune system, or surgery to remove an overactive thymus. Emergency treatment for a myasthenic crisis involves plasma exchange or intravenous immunoglobulins to rapidly remove autoantibodies and relieve symptoms.
Medications
The first-line treatment for myasthenia gravis is to increase the ability of nerves to pass signals to the muscles. Acetylcholinesterase inhibitors allow the neurotransmitter acetylcholine to linger at the junctions between nerves and muscles, giving the nerves more time to get signals through to the muscles. If these drugs do not control symptoms, patients will be put on drugs that dial back or suppress the immune system.
Surgery
The thymus, a small organ in the chest between your collar bones, produces immune system cells (lymphocytes) that fight infections and make antibodies such as the ones that cause myasthenia gravis. Problems with the thymus, such as cancer (thymoma) or overactive growth (hyperplasia), can produce an excess of immune cells and myasthenia gravis antibodies. In these cases, a thoracic surgeon will remove the thymus, a procedure known as thymectomy. This often produces complete and permanent symptom remission, but the effects may take months or years after the surgery to become noticeable.
Intravenous immunoglobulin (IVIg)
For a myasthenic crisis, surgical operations, or severe myasthenia gravis, an intravenous infusion of human antibodies (immunoglobulins) can rapidly reduce symptoms. These are essentially antibodies to the autoantibodies that cause myasthenia gravis. They attach to the harmful autoantibodies, and the immune system then clears them from the bloodstream. A single infusion may be necessary for a medical emergency or before an operation, but patients with severe myasthenia gravis will require regular infusions.
Plasmapheresis (PLEX)
Plasmapheresis “cleans” antibodies from the bloodstream. Also called plasma exchange (PLEX), a needle in one arm removes blood from the body. The blood is sent to a device that separates the blood cells from the plasma, the mix of water and proteins, including antibodies. The red blood cells are combined with new plasma without harmful antibodies, and the blood is returned to the patient. Plasmapheresis is usually used to treat a myasthenic crisis, but patients with uncontrolled symptoms may require regular plasma exchange.
Rehabilitation
Rehabilitation is a critical and often overlooked component of myasthenia gravis treatment. The goal is to relieve symptoms, improve daily function, and increase the quality of life. Physical therapy, strength training, respiratory therapy, occupational therapy, speech therapy, or vocational counseling can help bring back or maintain the ability to carry on everyday tasks.
Myasthenia gravis medications
First-line drugs for myasthenia gravis are intended to reduce symptoms. If they don’t work, however, second-line treatment focuses on suppressing the immune system. By suppressing the immune system, these medications attack the root of the problem: an overactive immune system.
Acetylcholinesterase inhibitors
Acetylcholinesterase inhibitors are the first-line treatment for myasthenia gravis. They relieve MG symptoms by increasing the amount of acetylcholine at the junctions between nerves and muscles. This increases muscle strength and reflex times but does not address the underlying autoimmune condition. The only acetylcholinesterase inhibitor approved for myasthenia gravis is pyridostigmine, but similar drugs may be prescribed if pyridostigmine does not sufficiently work. Symptoms noticeably improve in about 15 minutes and will last for three to four hours. However, their effectiveness depends on the type of autoantibodies responsible, so some patients may need higher doses.
Corticosteroids
When acetylcholinesterase inhibitors do not adequately relieve weakness and fatigue, the second-line drugs for myasthenia gravis are corticosteroids. Normally used to reduce swelling, corticosteroids also suppress the immune system and decrease the number of autoantibodies generated by immune cells. Oral or injected prednisone, prednisolone, or methylprednisolone are given in high doses that are steadily decreased to a maintenance dose over time.
Immune suppressants
Immunosuppressants are the next step if corticosteroids are not effective or their side effects are intolerable. Azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide, cyclosporine, and tacrolimus all work in different ways to either reduce the number of immune cells (lymphocytes) or prevent them from producing autoantibodies.
Monoclonal antibodies
Monoclonal antibodies are synthetic antibodies that attach to the surface of lymphocytes (immune cells). Once attached, they either stop lymphocytes from growing or block chemical activities at the neuromuscular junction that provoke the autoimmune processes (Soliris). Soliris (eculizumab) is well-studied and the only drug other than pyridostigmine and methotrexate that is FDA-approved for treating myasthenia gravis. Rituxan (rituximab) is either used alone or with other immune suppressants. Both significantly reduce symptoms over the long-term but are reserved for more severe cases that have not responded to other drugs.
What is the best medication for myasthenia gravis?
Myasthenia gravis is nicknamed the “snowflake disease” because it varies so much from person to person. Successful treatments will also vary between people, so there is no “best” medication for myasthenia gravis. The goal is to find a medication that provides dramatic or complete symptom relief with the fewest side effects. It may take a few tries to discover just the right medication that fits the patient’s situation.
| Best medications for myasthenia gravis | ||||
|---|---|---|---|---|
| Drug Name | Drug Class | Administration Route | Standard Dosage | Common Side Effects |
| Mestinon (pyridostigmine) | Acetylcholinesterase inhibitor | Oral | 600 mg to 1,500 mg divided into doses taken throughout the day | Sweating, diarrhea, muscle cramps |
| Bloxiverz (neostigmine) | Acetylcholinesterase inhibitor | Injection | 0.5 mg into the muscle or under the skin for symptomatic relief. Dose depends on weight in children and infants | Nausea, vomiting, low blood pressure |
| Deltasone (prednisone) | Corticosteroid | Oral | Dosage will vary. Initial dose will be high and tapered off until the lowest possible maintenance dose is reached | Behavior and mood changes, increased blood pressure, fluid retention |
| Orapred (prednisolone) | Corticosteroid | Oral | Dosage will vary. Initial dose will be high and tapered off until the lowest possible maintenance dose is reached | Behavior and mood changes, fluid retention, trouble sleeping |
| Imuran (azathioprine) | Immunosuppressant | Oral | Initially 50 mg once daily for one week. Daily maintenance dose depends on weight | Bone marrow suppression, abdominal pain, nausea |
| CellCept (mycophenolate mofetil) | Immunosuppressant | Oral | 1,000 mg to 1,250 mg twice daily | Bone marrow suppression, abdominal pain, nausea |
| Otrexup (methotrexate) | Immunosuppressant | Injection | Standard dosage not established for myasthenia gravis | Dizziness, headache, hair loss |
| Soliris (eculizumab) | Monoclonal antibody | Intravenous infusion | 900 mg every seven days for four weeks. Then, a single dose of 1,200 mg given seven days after the fourth dose. Then, 1,200 mg every 14 days. | Headache, back pain, nausea |
| Rituxan (rituximab) | Monoclonal antibody | Intravenous infusion | Dose depends on body surface area | Infusion reactions, low white blood cell counts, fever |
The standard dosages above are from the U.S. Food and Drug Administration (FDA) and the National Institute of Health (NIH). Dosage is determined by your doctor based on your medical condition, response to treatment, age, and weight. Other possible side effects exist. This is not a complete list
What are the common side effects of myasthenia gravis medication?
All medications may have side effects, and different classes of medications have different side effects. Most medications used to control myasthenia gravis are taken long-term, so side effects are more likely. However, this is not a complete list, and you should ask a healthcare professional about possible side effects and drug interactions based on your specific situation.
Anticholinesterase inhibitors increase both involuntary (muscarinic) and voluntary (nicotinic) muscle activity, so most side effects are related to one of these effects Muscarinic side effects include increased salivation, diarrhea, bowel cramping, bronchial secretions, and bronchial spasms. Nicotinic side effects include skeletal muscle cramping and twitching.
Corticosteroids typically cause increased appetite, weight gain, fluid retention, high blood pressure, acne, mood changes, and thinning skin. Because corticosteroids are used long-term to control myasthenia gravis, serious side effects, such as glaucoma, cataracts, high blood sugar, and osteoporosis, are possible. As an immunosuppressant, corticosteroids may raise the risk of infections.
Common side effects of immunosuppressive drugs include nausea, vomiting, and hair loss. Both immunosuppressants and immunotherapy antibodies weaken the immune system, so the most serious and sometimes life-threatening side effects are cancers and infections. Other severe side effects might include arthritis, organ damage, and, with monoclonal antibodies, severe allergic reactions.
What is the best home remedy for myasthenia gravis?
There is no cure for myasthenia gravis. Medications can help relieve or even eliminate symptoms, but lifestyle changes are a major component in long-term successful treatment.
Make your home safe
Your first priority is safety, so make sure your home is equipped to keep you safe during bouts of weakness or fatigue. Install safety devices such as raised bars in the bathroom and tub, corner protectors on wood furniture, and anti-slip surfaces or tape on the floor. Stairs should have hand railings, carpeting, and adequate lighting. Keep furniture and other obstacles out of the way of walking paths. Electrical cords should not run across floors. Finally, coffee tables and other low-slung furniture are hazardous no matter what a person’s health status is.
Make your home easy to live in
A few small changes can make homes easier to live in. Sofas and chairs should not be too low, making it difficult to get up. They should also have armrests to make it easier to get up or to act as support when standing. Objects that are often used, such as eyeglasses and keys, should be placed in a prominent, easy-to-access place all the time.
Make daily tasks easier
There is no award for making life tough. Electrical toothbrushes, can openers, and mixers reduce exertion and conserve your energy for other tasks. Make sure that cleaning tools such as sponges, brooms, and mops are located in one spot and easily accessible.
Make eating easier
Eating can be difficult and exhausting when living with myasthenia gravis. Plan your meals for times when you feel strongest. Eat several small meals a day and take breaks while eating. And, of course, eating soft foods that are easy to chew requires less exertion than tougher foods. Just in case, always keep a store of liquid meals that you can turn to when you’re feeling particularly fatigued.
Plan out your day
Patients with myasthenia gravis learn to plan their day. The goal is to schedule chores when you feel the strongest. However, planning also allows you to minimize effort. Simple things like making a shopping list can substantially reduce effort and exertion.
Reduce stress
Exacerbations are more likely during times of stress. Build regular stress-reducing routines into your day. Relaxing baths, yoga, and meditation can all help manage stress. Above all, maintain good sleep hygiene so you’re fully rested every day.
Strengthen your respiratory muscles
Don’t forget the most important muscles in your body, the ones that control breathing. In some cases, breathing can be compromised during exacerbations. Build in a regular respiratory muscle workout into your day. You can find many respiratory exercises and devices online.
Frequently asked questions about myasthenia gravis
Can myasthenia gravis go away?
There is no cure for myasthenia gravis. However, treatment can successfully reduce and even eliminate symptoms for months or years.
What is the best treatment for myasthenia gravis?
The goal of myasthenia gravis treatment is to reduce or eliminate symptoms. Doctors will start with cholinesterase inhibitors. These drugs only treat the symptoms, but their side effects are relatively moderate. If they don’t work, a healthcare provider will turn to drugs that modulate the immune system such as immunosuppressants, IVIg injections, plasmapheresis, and sometimes thymectomy, or the surgical removal of the thymus.
What is the life expectancy of a person with myasthenia gravis?
The life expectancy of patients with myasthenia gravis is not statistically different than the average human life expectancy.
What foods help myasthenia gravis?
As with all conditions, there are many claims that certain foods or nutrients “help” myasthenia gravis. While some people may experience symptom improvement by changing their diet, no food, nutrient, or diet has been proven to be clinically relevant in the treatment of myasthenia gravis. As with any health condition, the best diet is one that is healthy and rich in nutrients.
What foods should I avoid with myasthenia gravis?
Eating is a physically demanding activity, so some patients with myasthenia gravis may have difficulty getting through a meal. One way to make eating easier is to avoid foods that are hard to chew, like meats. It may be a good idea to keep liquid meals on hand for particularly bad bouts of fatigue or weakness.
What drugs are used for myasthenia gravis?
Myasthenia gravis is initially treated with drugs that address the symptoms. Anticholinesterase inhibitors increase the ability of nerves to communicate with muscles, increasing strength and endurance. If these drugs don’t work, doctors will turn to drugs that address the underlying problem: an overactive immune system. These drugs, called immune suppressants, dial back the ability of the immune system to attack the junctions between nerves and muscles. While effective, they leave patients vulnerable to infections and cancer.
What medications should not be taken with myasthenia gravis?
Any drug that interferes with the ability of nerves to communicate with muscle fibers will worsen myasthenia gravis symptoms and possibly provoke a myasthenic crisis. These include muscle relaxants, beta blockers, calcium channel blockers (a type of blood pressure medication), sodium channel blockers (a type of heart medication), antimalarial drugs, and some types of antibiotics. Botox, magnesium, lithium, and penicillamine are also contraindicated for myasthenia gravis. Patients with myasthenia gravis should be wary about taking drugs such as benzodiazepines, sedatives, antihistamines, opioids, anticonvulsants, antipsychotics, and nerve pain agents. These drugs also slow down the nervous system, and all have been known to cause a myasthenic crisis in some patients. Talk to your doctor if any of them are prescribed for you.
What natural treatment is good for myasthenia gravis?
Myasthenia gravis symptoms can be successfully managed in many patients, but the underlying condition cannot be cured. In a minority of patients, symptoms may not be manageable. It makes sense that some patients will turn to alternative or complementary medicines such as Chinese herbal medicines, acupuncture, massage, hydrotherapy, meditation, and biofeedback. Individual patients have achieved significant symptom relief with all these interventions, and some are incorporated into rehabilitation therapy. However, there is no solid evidence that any of these therapies are clinically effective.
How does pyridostigmine treat myasthenia gravis?
Pyridostigmine is often the first-line medical treatment for myasthenia gravis. As a cholinesterase inhibitor, it increases the length of time the neurotransmitter acetylcholine remains at the junction between nerves and muscle cells. It works by interfering with the enzyme, cholinesterase, which breaks down acetylcholine at the neuromuscular junctions. As a result, nerves have more time to pass signals to muscle cells, increasing the strength of the muscles, and reducing fatigue.
How do you reverse myasthenia gravis?
Myasthenia gravis is an autoimmune disease that cannot be cured. However, the muscle weakness and fatigue characteristic of the condition can be reversed with medical treatments including acetylcholinesterase inhibitors or immunosuppressive therapies.