Myasthenia gravis

Answer 1

Definition

Myasthenia gravis is a neuromuscular disorder. Neuromuscular disorders involve the muscles and the nerves that control them.

Alternative NamesNeuromusclar disorder - myasthenia gravis Causes, incidence, and risk factors

Myasthenia gravis causes weakness of the voluntary (skeletal) muscles. These are the muscles that create movement and are normally under your conscious control. The involuntary muscles, on the other hand, are not under conscious control (such as the muscles of your heart and many other internal organs).

In myasthenia gravis, weakness occurs because the nerve that activates a particular muscle does a poor job of stimulating that muscle. This problem occurs because immune cells (which normally attack foreign invaders) target and attack the body's own healthy cells. This is known as an autoimmune response. This autoimmune response produces antibodies that block the muscle cells from receiving messages (neurotransmitters) from the nerve cell.

The cause of autoimmune disorders such as myasthenia gravis is unknown. In some cases, myasthenia gravis may be associated with tumors of the thymus (an organ of the immune system). Patients with myasthenia gravis have a higher risk of having other autoimmune disorders, such as thyrotoxicosis, rheumatoid arthritis, and systemic lupus erythematosus (lupus).

Myasthenia gravis can affect people at any age. It is most common in young women and older men.

Symptoms

The muscle weakness of myasthenia gravis worsens with activity and improves with rest. Weakness in affected muscles may cause:

• Breathing difficulty because of weakness of the chest wall muscles
• Chewing or swallowing difficulty, causing frequent gagging, choking, or drooling
• Difficulty climbing stairs, lifting objects, or rising from a seated position
• Difficulty talking
• Drooping head
• Facial paralysis or weakness of the facial muscles
• Fatigue
• Hoarseness or changing voice
• Weakness of the eye muscles, causing
  • Double vision
  • Difficulty maintaining steady gaze
  • Eyelid drooping

Signs and tests

A neurological examination may be normal or may show muscle weakness that progressively worsens as the muscle is used. In many people with myasthenia gravis, the eye muscles are affected first. Reflexes and feeling (sensation) are normal. Weakness may affect the arms, legs, breathing or swallowing muscles, and any other muscle group.

Tests that may be performed:

• Acetylcholine receptor antibodies (may be present in the blood)
• CT or MRI scan of the chest (may show a tumor in the thymus)
• Repetitive stimulation (a type of nerve conduction study, may be more sensitive)
• Single-fiber EMG (may be very sensitive)

Treatment

There is no known cure for myasthenia gravis. However, treatment may result in prolonged periods without any symptoms (remission).

Lifestyle adjustments usually enables continuation of many activities. Activity should be planned to allow scheduled rest periods. An eye patch may be recommended if double vision is bothersome. Stress and excessive heat exposure should be avoided because they can worsen symptoms.

Some medications, such as neostigmine or pyridostigmine, improve the communication between the nerve and the muscle. Prednisone and other medications (such as azathioprine, cyclosporine, or mycophenolate mofetil) that suppress the autoimmune response responsible for the weakness may be used if symptoms are severe and other medications don't work well enough.

Plasmapheresis may reduce symptoms for 4 - 6 weeks and is often used before surgery. In this technique, the person's blood plasma (the clear part of the blood) containing the antibodies is removed from the body and replaced with donated, antibody-free plasma or with other intravenous fluids.

Intravenous immunoglobulin infusions may be as effective as plasmapheresis. In this technique, a large volume of a mixture of helpful antibodies is given directly into the bloodstream to blunt the effect of the autoimmune antibodies.

Surgical removal of the thymus (thymectomy) may result in permanent remission or less need for medicines, especially when there is a tumor present.

Patients with eye problems may try lens prisms to improve vision. Surgery may also be performed on the eye muscles.

Several medications may make symptoms worse and should be avoided. Therefore, it is always important to check with your doctor about the safety of a medication before taking it.

Crisis situations, where muscle weakness involves the breathing muscles, may occur without warning with under- or overuse of medications. These attacks seldom last longer than a few weeks. Hospitalization and assistance with breathing may be required during these attacks. Often plasmapheresis is used to help end the crisis.

Support Groups

The stress of illness can often be helped by joining support groups where members share common experiences and problems. See myasthenia gravis - support group.

Expectations (prognosis)

There is no cure, but long-term remission is possible. There may be minimal restriction on activity in many cases. People who have only eye symptoms (ocular myasthenia gravis), may develop generalized myasthenia over time.

Pregnancy is possible for a woman with myasthenia gravis but should be closely supervised. The baby may be temporarily weak and require medications for a few weeks after birth but usually does not develop the disorder.

Complications

• Complications of surgery
• Myasthenic crisis (breathing difficulty), may be life threatening
• Restrictions on lifestyle (possible)
• Side effects of medications (see the specific medication)

Calling your health care provider

Call your health care provider if you develop symptoms of myasthenia gravis.

Go to the emergency room or call the local emergency number (such as 911) if you have breathing difficulty or swallowing problems.

References

Benatar M, Kaminski HJ. Evidence report: the medical treatment of ocular myasthenia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2007;68(24):2144-9.

Vincent A, Newsom-Davis J. Disorders of neuromuscular transmission. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier. 2007: chap 448.

Zinman L, Ng E, Bril V. IV immunoglobulin in patients with myasthenia gravis: a randomized controlled trial. Neurology. 2007;68(11):837-41.

Answer 2

Definition

Myasthenia gravis is a neuromuscular disorder. Neuromuscular disorders involve the muscles and the nerves that control them.

Alternative NamesNeuromusclar disorder - myasthenia gravis Causes, incidence, and risk factors

Myasthenia gravis is a type of autoimmune disorders. An autoimmune disorder occurs when the immune system mistakenly attacks healthy tissue. In people with myasthemia gravis, the body produces antibodiesthat block the muscle cells from receiving messages (neurotransmitters) from the nerve cell.

The exact cause of myasthenia gravis is unknown. In some cases, it may be associated with tumors of the thymus (an organ of the immune system).

Myasthenia gravis can affect people at any age. It is most common in young women and older men.

Symptoms

Myasthenia gravis causes weakness of the voluntary (skeletal) muscles. Voluntary muscles are those that are under your control. In other words, you think about moving your arm, and it moves. The muscle weakness of myasthenia gravis worsens with activity and improves with rest.

The muscle weakness can lead to a variety of symptoms, including:

• Breathing difficulty because of weakness of the chest wall muscles
• Chewing or swallowing difficulty, causing frequent gagging, choking, or drooling
• Difficulty climbing stairs, lifting objects, or rising from a seated position
• Difficulty talking
• Drooping head
• Facial paralysis or weakness of the facial muscles
• Fatigue
• Hoarseness or changing voice
• Double vision
• Difficulty maintaining steady gaze
• Eyelid drooping

Signs and tests

The health care provider performs a physical exam, including a detailed nervous system (neurological) examination. This may show:

• Muscle weakness - eye muscles are usually affected first
• Reflexes and feeling (sensation) are normal.

Tests that may be done may include:

• Acetylcholine receptor antibodies associated with this disease
• CT or MRI scan of the chest to look for a tumor
• Nerve conduction studies
• EMG

Treatment

There is no known cure for myasthenia gravis. However, treatment may allow you to have prolonged periods without any symptoms (remission).

Lifestyle changes often help you continue your daily activities. The following may be recommended:

• Scheduling rest periods
• An eye patch is double vision is bothersome
• Avoiding stress and heat exposure, which can make symptoms worse

Medications that may be prescribed include:

• Neostigmine or pyridostigmine to improve the communication between the nerves and the muscles
• Prednisone and other medications (such as azathioprine, cyclosporine, or mycophenolate mofetil) if to suppress the immune system response, if you have severe symptoms and other medicines have not worked well

Several medications may make symptoms worse and should be avoided. Therefore, it is always important to check with your doctor about the safety of a medication before taking it.

Crisis situations, where muscle weakness involves the breathing muscles, may occur without warning with under- or overuse of medications. These attacks seldom last longer than a few weeks. Hospitalization and assistance with breathing may be required during these attacks. Often procedure called plasmapheresis is used to help end the crisis. In this technique, the clear part of the blood (plasma) containing the antibodies is removed from the body and replaced with donated, antibody-free plasma or with other fluids.

Plasmapheresis may also help reduce symptoms for 4 - 6 weeks and is often used before surgery. Intravenous immunoglobulin infusions may work as well as plasmapheresis. In this technique, a large amount of helpful antibodies are given directly into the bloodstream.

Surgery to remove the thymus (thymectomy) may result in permanent remission or less need for medicines, especially when there is a tumor present.

Patients with eye problems may try lens prisms to improve vision. Surgery may also be performed on the eye muscles.

Support Groups

The stress of illness can often be helped by joining support groups where members share common experiences and problems. See: Myasthenia gravis - support group

Expectations (prognosis)

There is no cure, but long-term remission is possible. You may have to restrict some daily activites. People who have only eye symptoms (ocular myasthenia gravis), may develop generalized myasthenia over time.

Pregnancy is possible for a woman with myasthenia gravis but should be closely supervised. The baby may be temporarily weak and require medications for a few weeks after birth but usually does not develop the disorder.

Complications

The condition may cause life-threatening breathing problems. This is called a myasthetnic crisis.

Patients with myasthenia gravis have a higher risk of having other autoimmune disorders, such as thyrotoxicosis, rheumatoid arthritis, and systemic lupus erythematosus (lupus).

Calling your health care provider

Call your health care provider if you develop symptoms of myasthenia gravis.

Go to the emergency room or call the local emergency number (such as 911) if you have breathing difficulty or swallowing problems.

References

Benatar M, Kaminski HJ. Evidence report: the medical treatment of ocular myasthenia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2007;68(24):2144-9.

Vincent A, Newsom-Davis J. Disorders of neuromuscular transmission. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier. 2007: chap 448.

Zinman L, Ng E, Bril V. IV immunoglobulin in patients with myasthenia gravis: a randomized controlled trial. Neurology. 2007;68(11):837-41.

Reviewed By

Review Date: 06/18/2011

Kevin Sheth, MD, Department of Neurology, University of Maryland School of Medicine, Baltimore, MD. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.