Schilder's Disease (Myelinoclastic Diffuse Sclerosis, Diffuse Sclerosis)

What is Schilder's Disease?

Schilder’s disease is a rare neurological disorder and is often confused with MS (multiple sclerosis). The main difference is that Schilder’s disease is not an autoimmune condition. This is a progressive disorder that affects the central nervous system and causes a broad spectrum of symptoms.

Schilder’s disease may also be referred to as:

• myelinoclastic diffuse sclerosis
• diffuse cerebral sclerosis

What causes Schilder’s disease?

The cause of Schilder’s disease is unknown. However, it is known that the disease causes damage to the myelin. Myelin is the brain matter responsible for protecting nerve cells. In cases of Schilder’s disease, myelin damage occurs in patches that grow in size. Many people develop the disease following an infection.

Schilder’s disease is considered a rare variant form of MS, but it is not an autoimmune disease like MS. Autoimmune conditions occur when the immune system mistakes healthy tissue for a threat and start to attack it.

Who is affected?

Schilder’s disease most commonly affects children and young adults. It is slightly more common in males than females. MS is more common in adults and it is diagnosed in more females than males.

How is Schilder’s disease diagnosed?

A diagnosis is usually made based on clinical symptoms and the findings of tests, which may include an MRI scan and EEG (electroencephalographic) tests. Tests are used to detect lesions (also known as plaques) that have a diameter of at least 2cm and rule out other conditions.

What are the symptoms?

The symptoms of Schilder’s disease are caused by damage to the protective myelin covering and include:

• difficulty remembering things and memory loss
• weakness and stiffness in the muscles
• decreased range of movement in the limbs
• impaired cognition (function declines with time)
• difficulty with coordination
• changes to vision
• hearing loss
• changes in bowel habits and impaired bowel function
• difficulty with walking

As the disease progresses, it can also cause:

• high blood pressure
• irregular heartbeat
• breathing difficulties
• personality and behavioural changes

How is Schilder’s disease treated?

There is no cure for Schilder’s disease and symptoms advance over time. However, there are treatments that can help to alleviate symptoms and offer support for the individual. These include medication to reduce swelling and muscle pain and therapies including physiotherapy, speech and language therapy and occupational therapy to improve movement, mobility and speech and support individuals at home.

What is the prognosis?

Schilder’s disease is progressive and symptoms get worse with time. In many cases, the outlook is poor and life expectancy may be shortened, especially if symptoms affect the major organs and cardiovascular health.