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Myasthenia gravis

Neuromuscular disorder - myasthenia gravis

 

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

Causes

 

Myasthenia gravis is a type of autoimmune disorder . An autoimmune disorder occurs when the immune system mistakenly attacks healthy tissue. Antibodies are proteins made by the body's immune system when it detects harmful substances. Antibodies may be produced when the immune system mistakenly considers healthy tissue a harmful substance, such as in the case of myasthenia gravis. In people with myasthenia gravis, the body produces antibodies that block the muscle cells from receiving messages (neurotransmitters) from the nerve cells.

The exact cause of myasthenia gravis is unknown. In some cases, it is linked to 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 muscles -- muscles that are under your control. Autonomic muscles of the heart and digestive tract are usually not affected. The muscle weakness of myasthenia gravis worsens with activity and improves with rest.

This 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 and eyelids
  • Facial paralysis or weakness of the facial muscles
  • Fatigue
  • Hoarseness or changing voice
  • Double vision
  • Difficulty maintaining steady gaze

 

Exams and Tests

 

The health care provider will perform a physical exam. This includes a detailed nervous system (neurological) examination. This may show:

  • Muscle weakness, with eye muscles usually affected first
  • Normal reflexes and feeling (sensation)

Tests that may be done include:

  • Acetylcholine receptor antibodies associated with this disease
  • CT or MRI scan of the chest to look for a tumor
  • Nerve conduction studies
  • EMG
  • Pulmonary function tests
  • Edrophonium test to see if this medicine reverses the symptoms for a short time

 

Treatment

 

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

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

  • Resting throughout the day
  • Using an eye patch if double vision is bothersome
  • Avoiding stress and heat exposure, which can make symptoms worse

Medicines that may be prescribed include:

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

Crisis situations are attacks of weakness of the breathing muscles. These attacks can occur without warning when either too much or too little medicine is taken. These attacks usually last no longer than a few weeks. You may need to be admitted to the hospital, where you may need breathing assistance with a ventilator .

A procedure called plasmapheresis may also be used to help end the crisis. This procedure involves removing the clear part of the blood (plasma), which contains the antibodies. This is replaced with donated plasma that is free of antibodies, or with other fluids. Plasmapheresis may also help reduce symptoms for 4 to 6 weeks and is often used before surgery.

Another procedure, called intravenous immunoglobulin (IVIg), may also be done. With this procedure, 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.

If you have eye problems, your doctor may suggest lens prisms to improve vision. Surgery may also be recommended to treat your eye muscles.

Physical therapy can help maintain your muscle strength. This is especially important for the muscles that support breathing.

Some medicines can worsen symptoms and should be avoided. Before taking any medicine, ask your doctor whether it is OK for you to take it.

 

Support Groups

 

You can ease the stress of illness by joining a myasthenia gravis support group . Sharing with others who have common experiences and problems can help you not feel alone.

 

Outlook (Prognosis)

 

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

A woman with myasthenia gravis can get pregnant, but careful prenatal care is important. The baby may be weak and require medications for a few weeks after birth, but usually will not develop the disorder.

 

Possible Complications

 

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

Patients with myasthenia gravis are at higher risk for other autoimmune disorders, such as thyrotoxicosis , rheumatoid arthritis , and systemic lupus erythematosus (lupus).

 

When to Contact a Medical Professional

 

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.

 

 

Open References

References

Meriggioli MN, Sanders DB. Disorders of neuromuscular transmission. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley’s Neurology in Clinical Practice . 6th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 78.

Mittal MK, Wijdicks EFM. Muscular paralysis. In: Parrillo JE, Dellinger RP, eds. Critical Care Medicine: Principles of Diagnosis and Management in the Adult . 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 64.

 
  • Superficial anterior muscles

    Superficial anterior muscles - illustration

    Superficial muscles are close to the surface of the skin. Muscles which lie closer to bone or internal organs are called deep muscles.

    Superficial anterior muscles

    illustration

  • Ptosis, drooping of the eyelid

    Ptosis, drooping of the eyelid - illustration

    Drooping of the eyelid is called ptosis. Ptosis may result from damage to the nerve that controls the muscles of the eyelid, problems with the muscle strength (as in myasthenia gravis), or from swelling of the lid.

    Ptosis, drooping of the eyelid

    illustration

    • Superficial anterior muscles

      Superficial anterior muscles - illustration

      Superficial muscles are close to the surface of the skin. Muscles which lie closer to bone or internal organs are called deep muscles.

      Superficial anterior muscles

      illustration

    • Ptosis, drooping of the eyelid

      Ptosis, drooping of the eyelid - illustration

      Drooping of the eyelid is called ptosis. Ptosis may result from damage to the nerve that controls the muscles of the eyelid, problems with the muscle strength (as in myasthenia gravis), or from swelling of the lid.

      Ptosis, drooping of the eyelid

      illustration

    A Closer Look

     

      Self Care

       

        Tests for Myasthenia gravis

         
         

        Review Date: 6/1/2015

        Reviewed By: Daniel Kantor, MD, Kantor Neurology, Coconut Creek, FL, and Immediate Past President of the Florida Society of Neurology (FSN). Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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