What is Schilder's Disease?

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Schilder's disease is a rare condition that usually starts in childhood. It's most common in boys between 7 and 12 years old.

It's a problem with the body's myelin.

Myelin is a protective layer that covers most of the nerves in your body. It's like the coating on electric wires. It helps signals move faster around your body, the way electricity flows from a power source. When the myelin is damaged, signals can't move the way they should.

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Schilder's disease is thought to be a form of multiple sclerosis. With MS, the immune system attacks the myelin, hurting it and the nerves it protects.

Schilder's disease has several other names. It's also known as:

  • Diffuse cerebral sclerosis
  • Diffuse cerebral sclerosis of Schilder
  • Myelinoclastic diffuse sclerosis

It's not the same as Addison-Schilder disease (adrenoleukodystrophy).

Causes

Doctors aren't sure what causes Schilder's disease. Because it usually starts when kids are so young, researchers think there may be a genetic link.

Symptoms

Schilder's disease often happens right after an infectious illness. It might start with an overall feeling of discomfort and fever.

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Later, symptoms might include:

  • Headache
  • Difficulty with speech
  • Personality changes
  • Vomiting
  • Attention issues
  • Problems with balance
  • Memory issues
  • Seizures
  • Tremors
  • Incontinence
  • Muscle weakness, particularly on one side of the body
  • Issues with hearing and vision
  • Irritability
  • Slowness of movement

As the disease gets worse, symptoms can get more serious. For example:

  • Gradual loss of awareness and responsiveness
  • Trouble with bowel and bladder control
  • Malnutrition
  • Respiration, blood pressure, and heart rate problems

Diagnosis

There's no specific test that can tell you if you have Schilder's disease. Your doctor will probably do a physical exam and ask you about your symptoms. You may also have tests done like an MRI and an EEG. With those, doctors hope to rule out other diseases that can cause similar symptoms. They're looking for specific scars on your myelin.

Treatment

The goal is to ease symptoms and keep your body working as smoothly as possible.

Your doctor may prescribe:

  • Corticosteroids
  • Beta interferon
  • Immunosuppressive drugs
  • Physiotherapy
  • Occupational therapy

Nutritional support through a dietitian could also be suggested.

There's no cure for Schilder's disease, but people respond differently to treatment. Some folks improve greatly, and the disease can go into remission.

Read more on: multiple sclerosis