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Myasthenia Gravis

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Please add people who have died as a result of Myasthenia Gravis, or a complication of it.

You may also add people who have been diagnosed with Myasthenia Gravis but may have survived &/or died of another cause.

Myasthenia gravis (MG) is a long-term, chronic autoimmune neuromuscular disease that causes weakness in the skeletal muscles, which are responsible for breathing and moving parts of the body, including the arms and legs.  The name myasthenia gravis, which is Latin and Greek in origin, means "grave, or serious, muscle weakness." The most commonly affected muscles are those of the eyes, face, and swallowing. It can result in double vision, drooping eyelids, trouble talking, and trouble walking. Onset can be sudden. Those affected often have a large thymus gland or develop a thymoma.

Myasthenia gravis is an autoimmune disease which results from antibodies that block or destroy nicotinic acetylcholine receptors at the junction between the nerve and muscle. This prevents nerve impulses from triggering muscle contractions. Rarely, an inherited genetic defect in the neuromuscular junction results in a similar condition known as congenital myasthenia. Babies of mothers with myasthenia may have symptoms during their first few months of life, known as neonatal myasthenia. Diagnosis can be supported by blood tests for specific antibodies, the edrophonium test, or nerve conduction studies.

Myasthenia gravis is generally treated with medications known as acetylcholinesterase inhibitors such as neostigmine and pyridostigmine. Immunosuppressants, such as prednisone or azathioprine, may also be used. The surgical removal of the thymus gland may improve symptoms in certain cases. Plasmapheresis and high dose intravenous immunoglobulin may be used during sudden flares of the condition. If the breathing muscles become significantly weak, mechanical ventilation may be required.

Myasthenia gravis affects 50 to 200 per million people. It is newly diagnosed in three to 30 per million people each year. Diagnosis is becoming more common due to increased awareness. It most commonly occurs in women under the age of 40 and in men over the age of 60. It is uncommon in children. With treatment, most of those affected lead relatively normal lives and have a normal life expectancy.

Symptoms:

  • Varying degrees muscle weakness, double vision, drooping eyelids, trouble talking, trouble walking
  • The initial, main symptom in MG is painless weakness of specific muscles, not fatigue. The muscle weakness becomes progressively worse during periods of physical activity, and improves after periods of rest. Typically, the weakness and fatigue are worse towards the end of the day. MG generally starts with ocular (eye) weakness; it might then progress to a more severe generalized form, characterized by weakness in the extremities or while performing basic life functions.
  • The hallmark of myasthenia gravis is muscle weakness that worsens after periods of activity and improves after periods of rest.  Certain muscles such as those that control eye and eyelid movement, facial expression, chewing, talking, and swallowing are often (but not always) involved in the disorder.  The muscles that control breathing and neck and limb movements may also be affected.

Prognosis

  • Diagnosis is often delayed because muscle weakness is a common symptom in other diseases and may slowly develop.
  • The prognosis of MG patients is generally good, as is quality of life, given very good treatment. In the early 1900s, the mortality associated with MG was 70%; now, that number is estimated to be around 3–5%, which is attributed to increased awareness and medications to manage symptoms.
  • Monitoring of a person with MG is very important, as at least 20% of people diagnosed with it will experience a myasthenic crisis within two years of their diagnosis, requiring emergent medical intervention. Generally, the most disabling period of MG might be years after the initial diagnosis.
  • With treatment, most individuals with myasthenia can significantly improve their muscle weakness and lead normal or nearly normal lives.  Sometimes the severe weakness of myasthenia gravis may cause respiratory failure, which requires immediate emergency medical care.
  • Some cases of myasthenia gravis may go into remission—either temporarily or permanently—and muscle weakness may disappear completely so that medications can be discontinued.  Stable, long-lasting complete remissions are the goal of thymectomy and may occur in about 50 percent of individuals who undergo this procedure.
  • The long-term outlook for MG depends on a lot of factors. Some people will only have mild symptoms. Others may eventually become confined to a wheelchair.
  • Early and proper treatment can limit disease progression in many people.

Epidemiology Myasthenia gravis occurs in all ethnic groups and both sexes.

  • The disease is not directly inherited nor is it contagious
  • It most commonly affects women under 40 and people from 50 to 70 years old of either sex, but it has been known to occur at any age. Younger patients rarely have thymoma.
  • It’s a relatively rare condition. The prevalence in the United States is estimated at between 14 and 20 out of every 100,000 people, with an estimated 60,000 Americans affected.
  • Within the United Kingdom, an estimated 15 cases of MG occur per 100,000 people.
  • According to the Myasthenia Gravis Foundation of America, MG is the most common primary disorder of neuromuscular transmission.
  • If a woman with myasthenia gravis gives birth, the baby may have some temporary, and potentially life-threatening, muscle weakness (neonatal myasthenia) because of antibodies that have transferred from the mother's bloodstream. Typically, during the baby's first weeks of life, the antibodies are cleared from the baby's circulation and the baby develops normal muscle tone and strength.

History

  • The first to write about MG were Thomas Willis, Samuel Wilks, Erb, and Goldflam. The term "myasthenia gravis pseudo-paralytica" was proposed in 1895 by Jolly, a German physician. Mary Walker treated a person with MG with physostigmine in 1934. Simpson and Nastuck detailed the autoimmune nature of the condition.[12] In 1973, Patrick and Lindstrom used rabbits to show that immunization with purified muscle-like acetylcholine receptors caused the development of MG-like symptoms.

Notable cases (Dx = diagnosed; CoD = cause of death)

  1. Wikipedia - Deaths from myasthenia gravis (7 listed)
  2. Famous People Diagnosed with Myasthenia Gravis (21 listed)

For Additional Reading:

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