What Are Demyelinating Disorders?

Most of the nerves in your body are covered with a protective layer called myelin. It’s a lot like the insulation on electric wires. It helps messages from your brain move quickly and smoothly through your body, like electricity flows from a power source.

Demyelinating disorders damage myelin. When this happens, scar tissue forms in its place. Brain signals can’t move across scar tissue as quickly, so your nerves don’t work as well as they should. As a result, you might have trouble talking, seeing, walking, and thinking.

Some types of these disorders happen because of your body’s reaction to a virus, or when your immune system attacks your own tissues, called an autoinflammatory response. But in many cases, doctors aren’t sure what causes these conditions.

The most common demyelinating disorder is multiple sclerosis (MS). One in 500 people have it. But there are other types.

Acute Disseminated Encephalomyelitis (ADEM)

ADEM is a brief but widespread bout of inflammation that can damage myelin in your brain, spinal cord, and sometimes the nerve connecting your eye to your brain, called the optic nerve. You get the condition when your body attacks its own tissues in response to infection with a virus or bacteria. Rarely, you can get it because of a reaction to a vaccine. Sometimes the cause is unknown.

Children get ADEM more often than adults do. Symptoms usually come on quickly. They include:

Most people recover fully within 6 months, though in very rare cases, ADEM can be deadly.

The best way to treat the disease is with drugs that fight inflammation. They can stop the damage to the nerves in the brain and spinal cord. A doctor also can prescribe other medicine to ease some ADEM symptoms.

Balo’s Disease (Concentric Sclerosis)

Some doctors think of Balo’s disease as a rare form of MS because the symptoms of both are the same in many ways. Experts don’t know why people get it, but it can cause serious problems. Some people can die from it, but it’s possible to recover fully, too.

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If you have Balo’s disease, your symptoms might come on quickly and get worse in a short amount of time, or they might go away quickly. Adults get the disease more often than children. Asians and people from the Philippines are the most likely to get it.

Symptoms include:

There isn’t a cure for Balo’s disease, and no drugs treat it. Your doctor can suggest medications to help with your symptoms, including corticosteroids to bring down the swelling in your brain and spinal cord.

HTLV-I Associated Myelopathy (HAM)

This condition is caused by a virus called HTLV-1. It can make your brain and spinal cord swell, which causes the symptoms of the disease. Not everyone who has the virus will get HAM. Some people also can carry HTLV-1 but have no symptoms.

People who get HAM usually live near the equator. You get it by coming into contact with blood or other body fluids of someone who has the disease. It’s not usually fatal, but you can die from it. Many people can live with the disease for decades.

Symptoms include:

There isn’t a cure for HAM, but there are medications you can take to help with symptoms, like corticosteroids.

Neuromyelitis Optica (Devic’s Disease)

This rare disease can affect your eyes, arms, and legs. Doctors don’t know what causes it, but they do know it makes your body attack your optic nerve and your spinal cord. You may have blurred vision or lose your eyesight. If it’s in your spinal cord, your legs and arms may not work well.

Most people who have one attack of neuromyelitis optica are likely to have another. But if you catch the disease early, your doctor will have a better chance to treat your symptoms. He may try using drugs that turn down your immune system to help keep you from having other relapses.

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Symptoms:

Neuromyelitis optica doesn’t have a cure or FDA-approved medications to treat it. Your doctor may give you a corticosteroid shot to help with swelling. He may also try a treatment that takes out some of the liquid part of your blood, called plasma, and replaces it with a man-made version. It’s called plasma exchange (PLEX).

Immunosuppressant medications such as azathioprine, methotrexate, mycophenolate and rituximab can be used to prevent further attacks.

Schilder’s Disease

Schilder’s disease is a rare condition that shows up most often in boys between ages 7 and 12. It wears away myelin in the brain and spinal cord. When the disease is severe, it affects breathing, heart rate, and blood pressure.

Doctors aren’t sure what causes Schilder’s disease, but it usually starts with an infection. Often, a headache and fever are the first symptoms.

It’s hard to predict how the disease will affect someone. Some people will have attacks of symptoms followed by times of recovery. For others, the disease slowly gets worse over time. Signs include:

  • Weakness on one side of the body
  • Slow movements
  • Seizures
  • Trouble speaking
  • Vision and hearing problems
  • Memory problems
  • Change in personality
  • Weight loss

There’s no cure for Schilder’s disease, but some people can manage their symptoms well with corticosteroids and drugs that turn down the immune system.

Transverse Myelitis

Transverse myelitis is a disorder of the spinal cord. It can cause symptoms in many places in your body, depending on where on your spinal cord you lose myelin. You might get the condition as a symptom of neuromyelitis optica. It also makes you more likely to be diagnosed with MS later on. There are about 1,400 new cases of transverse myelitis each year.

More women get the condition than men. It happens in both children and adults. About half of cases start after an infection, but doctors aren’t sure what causes the disorder. Some people have long-lasting effects from it, and others recover with no problems.

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Symptoms include:

  • Problems moving your legs
  • Bladder and bowel problems
  • Lower back pain
  • Muscle weakness
  • Sensitivity to touch
  • Tingling or numbness in your toes
  • Fatigue

There is no cure for transverse myelitis or FDA-approved medication to treat it. Your doctor may use corticosteroid shots or plasma exchange (PLEX) to make the swelling go down in your spinal cord and relieve some of your symptoms.

WebMD Medical Reference Reviewed by Neil Lava, MD on June 10, 2017

Sources

SOURCES:

National Multiple Sclerosis Society: “What is Myelin?” “Balo’s Disease,” “HTLV-I Associated Myelopathy (HAM),” “Neuromyelitis Optica (NMO),” “Schilder’s Disease,” “Transverse Myelitis.”

Love, S. Journal of Clinical Pathology, 2006.

Neuropathology: “Demyelinative Diseases.”

National Institute of Neurological Disorders and Stroke: “Acute Disseminated Encephalomyelitis,” “Tropical Spastic Paraparesis,” “Neuromyelitis Optica,” “Schilder’s Disease,” “Transverse Myelitis.”

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