Long-term Outcome of Schilder Disease Treated With Interferon-β

Pediatrics. 2019 Nov;144(5):e20190505. doi: 10.1542/peds.2019-0505.


Schilder disease, also termed diffuse myelinoclastic sclerosis, is characterized by a large demyelinating lesion involving 1 or both sides of the centrum semiovale of the cerebral hemispheres. It often presents with tumorlike features and poses a diagnostic challenge. Schilder disease can be monophasic or relapsing, and disease-modifying therapy for the latter scenario is largely empirical. Here, we report a 14-year-old girl with relapsing Schilder disease within 1 year after disease onset. She has been followed-up for nearly 10 years and remains in sustained remission ever since interferon-β therapy was prescribed after the second attack. In this case study, it is suggested that interferon-β may induce long-term remission in relapsing Schilder disease and is therefore worth considering in this regard.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Brain / diagnostic imaging*
  • Diffuse Cerebral Sclerosis of Schilder / diagnostic imaging
  • Diffuse Cerebral Sclerosis of Schilder / drug therapy*
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Humans
  • Immunologic Factors / therapeutic use*
  • Interferon beta-1a / therapeutic use*
  • Magnetic Resonance Imaging
  • Methylprednisolone / therapeutic use
  • Neuroimaging
  • Neuroprotective Agents / therapeutic use
  • Remission Induction
  • Tomography, X-Ray Computed


  • Adrenal Cortex Hormones
  • Immunologic Factors
  • Neuroprotective Agents
  • Methylprednisolone
  • Interferon beta-1a