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Myasthenia Gravis (MG) Myasthenia Gravis (MG)


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is myasthenia gravis an autoimmune disorder?

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Myasthenia gravis is considered to be an autoimmune disorder. In an autoimmune disease, your immune system mistakes a part of your own body as foreign, resulting in its destruction. In the case of myasthenia gravis, your immune system targets the acetylcholine receptors that your muscles need in order to contract.

From:
Understanding Myasthenia Gravis — the Basics
WebMD Medical Reference

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Reviewed
by
Neil Lava
on
March 10, 2018

Medically

Reviewed

on
3/10/2018

SOURCES:  

National Institute of Neurological Disorders and Stroke.

Myasthenia Gravis Foundation of America.

Reviewed
by
Neil Lava
on
March 10, 2018

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SOURCES:  

National Institute of Neurological Disorders and Stroke.

Myasthenia Gravis Foundation of America.

Reviewed
by
Neil Lava
on
March 10, 2018
Who is commonly affected by myasthenia gravis?

NEXT QUESTION:

What percentage of people with myasthenia gravis patients have thymoma?

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    Myasthenia Gravis

    Medically reviewed by Deborah Weatherspoon, PhD, RN, CRNA on April 23, 2018 — Written by Jaime Herndon

    Myasthenia gravis

    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.

    What
    are the symptoms of myasthenia gravis?

    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, the communication between nerve and muscle is blocked 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 due to muscle weakness
    • difficulty swallowing or chewing
    • fatigue
    • hoarse voice
    • drooping of eyelids
    • double vision

    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.

    What
    causes myasthenia gravis?

    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 neuromuscular junction. Damage to the neuromuscular membrane reduces the effect of 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.

    How
    is myasthenia gravis diagnosed?

    Your doctor will 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, 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
    options for myasthenia gravis

    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.

    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.

    Complications
    of myasthenia gravis

    One of the most dangerous potential complications of MG is myasthenic crisis. This consists of life-threatening muscle weakness that can include breathing problems. Talk with your doctor about your risks. If you start to have trouble breathing or swallowing, call 911 or go to your local emergency room immediately.

    Individuals with MG are at a higher risk of developing other autoimmune disorders such as lupus and rheumatoid arthritis .

    Long-term
    outlook

    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. Talk to your doctor about what you can do to minimize the severity of your MG. Early and proper treatment can limit disease progression in many people.

    Medically reviewed by Deborah Weatherspoon, PhD, RN, CRNA on April 23, 2018 — Written by Jaime Herndon

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    • Neurology

    Autoimmune Myasthenia Gravis in Children

    Autoimmune myasthenia gravis (MG) is a complex, autoimmune disorder in which antibodies destroy neuromuscular connections. This causes problems with the nerves that communicate with muscles, resulting in weakness of the skeletal muscles. MG affects the voluntary muscles of the body, especially the eyes, mouth, throat, and limbs.

    Patients can be seen by Texas Children’s experts in Neurology .

    Causes & Risk Factors

    There are different forms of myasthenia gravis.  Autoimmune myasthenia gravis is not inherited as a rare genetic disease or contagious, but it may be acquired through immune proteins or antibodies to babies born to mothers with MG, or the disorder may develop spontaneously later in childhood.  Rare forms of myasthenia gravis are related to underlying genetic defects inherited from the parents.

    Symptoms & Types

    There are three types of MG in children, including the following:

    • Congenital MG. This is a very rare nonimmune form of MG that is most often inherited as an autosomal recessive disease. This means that both males and females are equally affected and that 2 copies of the gene, one inherited from each parent, are necessary to have the condition. Symptoms of congenital MG usually begin in the baby’s first year and are lifelong.
    • Transient neonatal MG. Babies born to mothers with autoimmune MG may have a temporary form of MG. This occurs when antibodies common in MG cross the placenta to the developing fetus. Neonatal MG usually lasts only a few weeks, and babies are not at greater risk for developing MG later in life.
    • Juvenile Autoimmune MG. This autoimmune disorder develops typically in female adolescents–especially Caucasian females. Rarely, it may develop in children under the age of 10 years. It is potentially a lifelong condition that may go in and out of remission. About 10% of MG cases are juvenile-onset.

    What are the symptoms of myasthenia gravis?

    The following are the most common symptoms of myasthenia gravis. However, each child may experience symptoms differently. Symptoms may include:

    • Babies with neonatal MG may be weak with a poor suck, and may have respiratory difficulty. A few babies may need the help of a mechanical breathing machine if their respiratory muscles are too weak to breathe on their own. Symptoms go away as the maternal antibodies disappear over time.
    • Congenital MG symptoms may begin in the first year, with generalized weakness in the arms and legs, and delays in motor skills such as crawling, sitting, and walking. Babies may have difficulty feeding and may have weak eyelids and poor head control.
    • Juvenile MG symptoms may begin gradually over weeks or months. The child may become excessively tired after very little activity, and begin to have problems chewing and swallowing. Drooping eyelids and lazy eye movements can cause vision problems.
    • Patients with myasthenia gravis can have attacks of increased weakness, even if they are receiving treatment. Such attacks require emergent medical evaluation.

    The symptoms of myasthenia gravis may resemble other conditions. Always consult your child’s doctor for a diagnosis.

    Diagnosis & Tests

    The diagnosis of myasthenia gravis is made after the sudden or gradual onset of specific symptoms and after diagnostic testing. During the physical examination, the doctor obtains a complete medical history of the child and may also ask if there is a family history of any medical problems.

    A mainstay of the diagnosis of MG is through the performance of an electromyogram/nerve conduction study (EMG/NCS), which is a test that measures the electrical activity of a muscle or a group of muscles.   In addition, patients have a blood test for the antibodies associated with autoimmune MG.  An EMG/NCS can detect abnormal electrical muscle activity due to diseases and neuromuscular conditions. The diagnosis of MG may be confirmed with a Tensilon or Edrophonium test. With this test, a small amount of medicine (Tensilon or edrophonium chloride) is injected through an IV. If the child has MG, an immediate, but brief, increase in muscle strength is noted.

    Other diagnostic tests that may be performed to help confirm the diagnosis of myasthenia gravis include:

    • Blood tests
    • Genetic tests. Diagnostic tests that evaluate for conditions that have a tendency to run in families.

    Treatment & Care

    Specific treatment for myasthenia gravis will be determined by your child’s doctor based on:

    • Your child’s age, overall health, and medical history
    • The extent of the condition
    • Your child’s tolerance for specific medications, procedures, or therapies
    • Expectations for the course of the condition
    • Your opinion or preference

    There is no cure for MG, but the symptoms can sometimes be controlled. Myasthenia gravis can be a lifelong medical condition, and the key to medically managing MG is early detection.

    The goal of treatment is to alleviate signs and symptoms of weakness and to minimize the possibility of acute attacks of increased weakness, which can affect swallowing and breathing.

    Treatment may include:

    • Medications (cholinesterase inhibitors, steroids, and immunosuppressants)
    • Thymectomy. This is the surgical removal of the thymus gland. The role of the thymus gland in MG is not fully understood, and the thymectomy may or may not improve a child’s symptoms.
    • High-dose intravenous immune globulin
    • Plasmapheresis. A procedure that removes abnormal antibodies from the blood and replaces the child’s blood with normal antibodies through donated blood.

    The extent of the problems is dependent on the severity of the condition and the presence of other problems that could affect the child. In severe attacks, a breathing machine may be required to help the child breathe easier.

    The health care team educates the family after evaluation and diagnosis on how to best care for their child at home and outlines specific clinical problems that require immediate medical attention by their doctor. A child with MG requires frequent medical evaluations throughout his life.

    It is important to allow the child as much independent function and self care, especially with juvenile MG, as possible and to promote age-appropriate activities to ensure a sense of normalcy.