Schilders Disease

Schilder’s disease is a rare progressive demyelinating disorder (the myelin sheath of neurons are damaged) which usually begins in childhood. It is also referred to as diffuse myelinoclastic sclerosis.

The onset of the disease is typically in children between 5 and 14 years old, but cases in adults are also possible.

Symptoms may include:

  • Dementia
  • Aphasia – a language disorder
  • Seizures
  • Personality changes
  • Poor attention
  • Tremors
  • Balance instability
  • Incontinence
  • Muscle weakness
  • Headache
  • Vomiting
  • Vision and speech impairment

Schilder’s disease is a variant of multiple sclerosis. It is not the same as Addison-Schilder disease (adrenoleukodystrophy).

The criteria for diagnosis are:

  • One or two roughly symmetrical large plaques. Plaques are greater than 2 cm diameter.
  • No other lesions are present and there are no abnormalities of the peripheral nervous system.
  • Pathological analysis is consistent with subacute or chronic myelinoclastic diffuse sclerosis.
  • Results of adrenal function studies and serum very long chain fatty acids are normal.

As with multiple sclerosis, the course and prognosis of Schilder’s disease are unpredictable. For some individuals the disorder is progressive with a steady, unremitting course. Others may experience significant improvement and even remission. In some cases, Schilder’s disease is fatal.

Treatment for the disorder follows the established standards in multiple sclerosis and includes corticosteroids, beta-interferon or immunosuppressive therapy, and symptomatic treatment.