Myasthenia Gravis (MG)

Myasthenia Gravis (MG) in Children

What is myasthenia gravis in children?

Myasthenia gravis (MG) is a disorder that causes weakness in muscles around the body. This happens because antibodies destroy some of the places where nerves and muscles meet (neuromuscular connections). MG affects the voluntary muscles of the body. It mostly affects the eyes, mouth, throat, arms, and legs. It most often affects adults, but it can sometimes happen in children.

There are 3 types of MG in children:

Transient neonatal MG

This is a temporary form of MG. It can happen in babies born to mothers with MG. It occurs when antibodies from MG cross over to the baby growing in the mother’s uterus. Transient neonatal MG usually lasts only a short time. The symptoms go away weeks or months after birth, as the antibodies are naturally replaced. Babies are not at greater risk of developing MG later in life.

Juvenile MG

This is an autoimmune disorder. It most often occurs in white teen girls. It is a lifelong condition that may go in and out of remission. Treatment is often done by removing the entire thymus gland. This may cure the disorder.

Congenital myasthenic syndromes

These are rare hereditary forms of myasthenia, inherited as an autosomal recessive disease. This means that both boys and girls are equally affected. It also means that 2 copies of the gene, 1 inherited from each parent, are needed to have the condition. There are about 12 rare genetic disorders that may cause congenital MG. Symptoms of congenital MG usually start at birth or childhood and are lifelong. Some forms may be treated with the same medicines that are used for adults. Because these aren't autoimmune conditions, immunosuppression isn't usually helpful. These conditions collectively are called myasthenic syndromes.

What causes myasthenia gravis in a child?

MG is not contagious. This means it can’t be spread from person to person. It may be gotten through immune proteins or antibodies in babies born to mothers with MG. Or the disorder may develop by chance later in childhood. Rare forms of MG are caused by gene changes.

Which children are at risk for myasthenia gravis?

A child is more at risk for myasthenia gravis if they have a mother with MG, or parents with certain gene changes.

What are the symptoms of myasthenia gravis in a child?

Symptoms can occur a bit differently in each child, and they vary by type of MG. They can include:

Transient neonatal MG

Babies may have a weak cry, poor sucking, and generalized weakness. They may have breathing problems. In some cases, a baby’s respiratory muscles are so weak the baby can’t breathe on their own. Then the baby may need the help of a mechanical breathing machine (ventilator). Symptoms often go away as the mother’s antibodies disappear over time.

Juvenile MG

Symptoms may start slowly over weeks or months. A child may become very tired after very little activity. They may start to have trouble with chewing and swallowing. Drooping eyelids may be so bad that the child can’t see or may have double vision. The symptoms tend to be milder in the morning and worse later in the day or after exercise.

Congenital myasthenic syndromes

The symptoms are usually noted at birth, but they may begin in childhood. They include overall weakness in the arms and legs. Babies may have delays in motor skills such as crawling, sitting, and walking. They may have trouble feeding or breathing. And they may have weak eyelids and poor head control.

The symptoms of MG can be like other health conditions. Make sure your child sees their healthcare provider for a diagnosis.

How is myasthenia gravis diagnosed in a child?

The healthcare provider will ask about your child’s symptoms and health history. They may also ask about your family’s health history. They will give your child a physical exam. Your child may also have tests, such as:

• Electromyogram/nerve conduction study (EMG/NCS). This is a test that measures the electrical activity of a muscle or a group of muscles. This is the main way to diagnose MG. An EMG/NCS can find abnormal electrical muscle activity. In some cases, a single-fiber EMG test may be done. This is a more sensitive test. It may not be done in as many healthcare centers.
• Tensilon test. Your child may be given a medicine called an anticholinesterase inhibitor, such as neostigmine or pyridostigmine. It may be given by mouth or injected. In some cases, the medicine is given during the EMG test.
• Blood tests. These check for certain kinds of antibodies in the blood.
• Genetic tests. These tests look for conditions that tend to run in families.

How is myasthenia gravis treated in a child?

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on the severity of the condition and the type of MG.

Transient neonatal MG goes away on its own in weeks or months. There is no cure for juvenile or congenital MG, but the symptoms may be controlled. It is usually a lifelong condition.

A child may need:

• Breathing support. Your child may need a mechanical breathing machine (ventilator) to breathe.
• Nutritional support. If your child has swallowing problems, they may need tube feeding or other care.

Treatment may also include any of these:

• Medicines. These include cholinesterase inhibitors, steroids, and immunosuppressants.
• Thymectomy. This is surgery to remove the thymus gland. The role of the thymus gland in autoimmune MG is not fully understood. A thymectomy may or may not improve a child's symptoms or cure MG. The benefits of a thymectomy in children with MG are not as proven as in adults with MG. The results of the antibody tests may help your child’s healthcare provider evaluate if a thymectomy may help.
• High-dose intravenous immune globulin. This is a procedure to add a large amount of normal antibodies from donated blood into the child’s bloodstream.
• Plasmapheresis. This is a procedure to remove abnormal antibodies from the blood and replace them with normal antibodies from donated blood.

Talk with your child’s healthcare providers about the risks, benefits, and possible side effects of all treatments.

What are possible complications of myasthenia gravis in a child?

In severe cases, there can be life-threatening weakness of the breathing or swallowing muscles. This is called crisis. A child may need a breathing machine (ventilator) to help with breathing during crises.

How can I help prevent myasthenia gravis in my child?

Talk with your healthcare provider if you are pregnant and have MG. Your healthcare provider may advise genetic counseling. You can learn more about the risk of MG in a future pregnancy.

How can I help my child live with myasthenia gravis?

Issues may come up as a child grows and develops. Children with MG need to see their healthcare team often.

You can help your child strengthen their self-confidence and be as independent as possible. The healthcare team will talk with you about the best ways to help your child.

When should I call my child’s healthcare provider?

Call the healthcare provider right away if your child has:

• Symptoms that don’t get better, or get worse
• New symptoms

Key points about myasthenia gravis in children

• Myasthenia gravis (MG) is a disorder that causes weakness in muscles around the body. It mostly affects the eyes, mouth, throat, arms, and legs.
• Transient neonatal MG is a temporary form of MG. It can happen in babies born to mothers with MG. The symptoms usually go away weeks or months after birth.
• Juvenile MG is an autoimmune disorder. It is a lifelong condition that may go in and out of remission.
• Congenital myasthenic syndromes are very rare forms of myasthenia that are inherited. Symptoms usually start at birth and are lifelong.
• Treatment may include medicines, surgery, or blood product infusions. In severe cases, a child may need a breathing machine (ventilator) to help with breathing.
• Issues may come up as a child grows and develops. Children with MG need to see their healthcare team often.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.
• Before your visit, write down questions you want answered.
• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are and when they should be reported.
• Ask if your child’s condition can be treated in other ways.
• Know why a test or procedure is advised and what the results could mean.
• Know what to expect if your child doesn't take the medicine or have the test or procedure.
• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your child’s healthcare provider after office hours. This is important if your child becomes ill and you have questions or need advice.