Myasthenia Gravis

Highlights
• MG is rare and only affects 14 to 20 people
out of every 100,000.
• Treatment for MG consists of symptom
management.
• MG typically occurs in individuals over the age
of 40.
 Definition
• Myasthenia gravis (MG) is a neuromuscular disorder
that causes weakness in the skeletal muscles, which
are the muscles your body uses for movement. It
occurs when communication between nerve cells and
muscles becomes impaired. This impairment prevents
crucial muscle contractions from occurring, resulting in
muscle weakness. According to the Myasthenia Gravis
Foundation of America, MG is the most common
primary disorder of neuromuscular transmission. It’s a
relatively rare condition that affects between 14 and 20
out of every 100,000 people in the United States.
 Symptoms
• The main symptom of MG is weakness in
the voluntary skeletal muscles, which are
muscles under your control. The failure of
muscles to contract normally occurs
because they can’t respond to nerve
impulses. Without proper transmission of
the impulse, a blocked communication
occurs between nerve and muscle and
weakness results.
Weakness associated with MG typically gets worse with more
activity and improves with rest. Symptoms of MG can include:
• trouble talking
• problems walking up stairs or lifting objects
• facial paralysis
• difficulty breathing because of muscle weakness
• difficulty swallowing or chewing
• fatigue
• hoarse voice
• drooping of eyelids (Ptosis (often the most common initial
symptom))
• double vision (Diplopia)
Not everyone will have every symptom, and the degree of
muscle weakness can change from day to day. The severity of
the symptoms typically increases over time if left untreated.
 Causes
• MG is a neuromuscular disorder that’s usually
caused by an autoimmune problem. Autoimmune
disorders occur when your immune system
mistakenly attacks healthy tissue. In this condition,
antibodies, which are proteins that normally
attack foreign, harmful substances in the body,
attack the neurotransmitter substance
acetylcholine, which is a crucial substance for
communication between nerve cells and muscles.
This results in muscle weakness.
• The exact cause of this autoimmune reaction is
unclear to scientists. According to
the Muscular Dystrophy Association, one
theory is that certain viral or bacterial proteins
may prompt the body to attack acetylcholine.
• According to the National Institutes of Health,
MG typically occurs in people over the age of
40. Women are more likely to be diagnosed as
younger adults, whereas men are more likely
to be diagnosed at 60 or older.
 Diagnosis
perform a complete physical exam, as well as take a
detailed history of your symptoms. They’ll also do a
neurological exam. This may consist of:
• checking your reflexes
• looking for muscle weakness
• checking for muscle tone
• making certain your eyes move properly
• testing sensation in different areas of your body
• testing motor functions, like touching your finger
to your nose
Other tests that can help your doctor diagnose the
condition include:
• repetitive nerve stimulation test
• blood testing for antibodies associated with MG
• edrophonium (Tensilon) test: A drug called
Tensilon (or a placebo) is administered
intravenously, through a vein, and you’re asked to
perform muscle movements under doctor
observation
• imaging of the chest, using CT scans or MRI, to
rule out a tumor
 Treatment
There is no cure for MG. The goal of treatment is to
manage symptoms and control the activity of your
immune system.
Medication
• Corticosteroids and immunosuppressants can be used
to suppress the immune system. These medications
help minimize the abnormal immune response that
occurs in MG.
• Additionally, cholinesterase inhibitors, such
as pyridostigmine (Mestinon), can be used to increase
communication between nerves and muscles.
 Thymus Gland Removal
• Removal of the thymus gland, which is part of the
immune system, may be appropriate for many
patients with MG. Once the thymus is removed,
patients typically show less muscle weakness.
• According to the Myasthenia Gravis Foundation
of America, between 10 and 15 percent of people
with MG will have a tumor in their thymus.
Tumors, even those that are benign, are always
removed because they may become cancerous.
 Role of the thymus gland in myasthenia gravis
• The thymus gland, which lies in the chest area beneath the breastbone,
plays an important role in the development of the immune system in
early life. Its cells form a part of the body's normal immune system.
The gland is somewhat large in infants, grows gradually until puberty,
and then gets smaller and is replaced by fat with age. In adults with
myasthenia gravis, the thymus gland remains large and is abnormal. It
contains certain clusters of immune cells indicative of lymphoid
hyperplasia—a condition usually found only in the spleen and lymph
nodes during an active immune response. Some individuals with
myasthenia gravis develop thymomas (tumors of the thymus gland).
Thymomas are generally benign, but they can become malignant.
• The relationship between the thymus gland and myasthenia gravis is
not yet fully understood. Scientists believe the thymus gland may give
incorrect instructions to developing immune cells, ultimately resulting
in autoimmunity and the production of the acetylcholine receptor
antibodies, thereby setting the stage for the attack on neuromuscular
transmission.
 Plasma Exchange
• Plasmapheresis is also known as a plasma
exchange. This process removes harmful
antibodies from the blood, which may result
in an improvement in muscle strength.
• Plasmapheresis is a short-term treatment. The
body continues to produce the harmful
antibodies and weakness may recur. Plasma
exchange is helpful before surgery or during
times of extreme MG weakness.
 Intravenous Immune Globulin
• Intravenous immune globulin (IVIG) is blood
product that comes from donors. It’s used to
treat autoimmune MG. Although it’s not
entirely known how IVIG works, it affects the
creation and function of antibodies.
 Lifestyle Changes
• There are some things you can do at home to help
alleviate symptoms of MG:
• Get plenty of rest to help minimize muscle weakness.
• If you’re bothered by double vision, talk to your doctor
about whether you should wear an eye patch.
• Avoid stress and heat exposure, as both can worsen
symptoms.
• These treatments cannot cure MG. However, you’ll
typically see improvements in your symptoms. Some
individuals may go into remission, during which
treatment is not necessary.
• Tell your doctor about any medications or supplements
you take. Some drugs can make MG symptoms worse.
Before taking any new medication, check with your
doctor to ensure it’s safe.
 Nursing Interventions
• Listen to the patient’s concerns and answer the questions honestly.
• Administer medications on time and at evenly spaced intervals, as ordered, to
prevent relapses.
• Plan exercise, meals, patient care, and activities to make the most of energy
peaks.
• When swallowing is difficult, give semi-solid foods instead of liquids to lessen
the risk of choking.
• After severe exacerbations, try to increase social activity as soon as possible.
• Establish accurate neurologic and respiratory baseline.
• Stay alert for signs of impending myesthenic crisis such as increased muscle
weakness and difficulty talking or chewing.
• Help the patient plan daily activities to coincide with energy peaks.
• Stress the need for frequent rest periods.
• If surgery is scheduled, provide perioperative teaching.