Myasthenia gravis

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Myasthenia Gravis

Definition:

A disorder characterized by chronic weakness of voluntary muscles, which

improves with rest and worsens with activity.

Causes, incidence, and risk factors:

Myasthenia gravis (MG) involves fluctuating levels of weakness of commonly

used muscles. It may affect any of the voluntary muscles. This is

confusing to everyone around the patient, as she/he may be fine one minute

and in difficulty the next.

Weakness occurs when the nerve impulse does not adequately reach the muscle

cells; this is caused by blockage of the action of transmitters (the

chemicals that transmit signals from nerve cell to muscle cell). This is

thought to be the result of an immune response of the body against itself

(an autoimmune response). High levels of antibodies block the action of

specific chemicals that are used to transmit messages from the nerve cell to

the muscle cell (neurotransmitters).

The cause of autoimmune disorders such as myasthenia gravis is unknown.

Myasthenia gravis affects about 3 out of 10,000 people. Except when the

disorder is associated with thymus tumor (which is most common in elderly

men), myasthenia gravis is most common in adult women. Symptoms may worsen

with stress, pregnancy or menstrual periods.

Prevention:

There is no known way to prevent this disorder.

Symptoms:

Vision changes:

Double vision

Difficulty maintaining steady gaze

Eyelid drooping or closing

Eyeglass prescription may fluctuate frequently

Swallowing difficulty, frequent gagging or choking (may be forced to take

only thickened liquids. No crumbly, dry foods. For some

unknown reason, water is often the hardest substance to swallow.)

Sagging of throat may cause food or liquids to go " down the wrong

pipe " .

Dental care can be a problem, as it is difficult to close off the

throat.

Lying on the back may cause muscles to sag and interfere

with breathing.

Hoarseness or changing voice

Weakness or paralysis that worsens with exertion later in the day

Facial paralysis (difficulty smiling, subject appears angry or tired)

Breathing, absent temporarily

Sustained breathing difficulty

Drooping head (can cause severe pain in neck and upper back)

Poor posture, slumping, leading to chest pains

Difficulty climbing stairs

Difficulty lifting objects

Need to use hands to arise from sitting or recumbent positions

Difficulty talking (slurring words, sounding drunk)

Difficulty producing the right words when needed (mind blank) and a

tendency to switch words, for instance saying right when the patient

means left (these symptoms are probably due to exhaustion.

It is not thought that the disease effects the brain in any direct way.)

Difficulty chewing

Fatigue

Overall weakness, sometimes to the point of collapse and/or paralysis

Weakness or paralysis improves temporarily with rest

Muscle function best after rest

Muscle function worst at the end of the day

Any sustained exertion (shopping, cleaning, walking, talking, dancing,

driving, etc.), may cause muscle weakness and fatigue, even collapse.

Weak muscle tone

Constantly watering eyes due to weakness of eyelid muscles

Muscle pain (develops because the muscles are not working correctly, so

even normal work is, for those muscles, overwork)

Swelling, overall

Muscle atrophy

Muscle cramps

Drooling

Fluctuating hearing (sound levels change)

Frequent clumsiness - running into walls, dropping things, stumbling,

tripping over things, falling

Tired, " heavy " , arms and/or legs

Lifestyle adjustments may enable continuation of many activities. Most

patients will have to curtail some former activity. The remaining activities

should be planned to allow scheduled rest periods. An eye patch may be

recommended if double vision is bothersome. Stress should be avoided,

because it worsens symptoms.

Crisis situations, where muscle weakness involves the breathing muscles, may

occur. These attacks seldom last longer than a few weeks. Hospitalization

and assistance with breathing may be required during these attacks.

Patient may collapse and be unable to move. The patient is awake and

perfectly aware of everything around them - just unable to respond.

Expectations (prognosis):

There is no cure, but long-term remission is possible. There may be minimal

restriction on activity in many cases. Cases vary a great deal, with some

patients on permanent disability and many able to function almost normally.

Complications:

restrictions on lifestyle

side effects of medications (See the specific medication.)

complications of surgery or other medical procedures

Myasthenic crisis (breathing difficulty), may be life threatening

Many drugs which are commonly given for breathing difficulties in an

emergency situation only make the Myasthenic worse. The

patient should carry a list of these drugs with him/her at all times. For

the same reason, it is a good idea to wear a Medic Alert bracelet or

necklace.

What to do if someone you know has Myasthenia gravis?

Learn about the disorder - be aware of its symptoms.

Offer help to the individual if help seems needed, but respect her/his

desire to be independent.

Understand that the symptoms fluctuate. The individual may be eager to

participate in a function one day and unable to the next.

Recognize that a grim-faced Myasthenic may be " smiling at you on the

inside " , but not able to show it on the outside.

If breathing problems develop and the individual is unable to cope, call

911. Be sure to tell the Medics that the individual has Myasthenia gravis

and that this disorder has many counter-indicated drugs.

(wsm311@...)

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