Inflammatory myelinoclastic diffuse sclerosis (Schilder's disease): Neuroradiologic findings

Mark F. Mehler, L. Rabinowich

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

This case and a review of the literature, suggest that, rigorously defined, IMDS is a subacute or chronic inflammatory myelinoclastic disorder resulting in extensive, often cavitary, bilateral hemispheric white-matter lesions with mass effect, the absence of noncontiguous foci, and histologic resemblance to acute multiple sclerosis (i.e., inflammatory demyelination with exuberant myelin regeneration). Diagnostic dilemmas in the field of demyelinating disease have directly resulted from the inconsistencies of Schilder's early reports and the absence of definitive biochemical analysis prior to 1980. Neuroimaging is crucial in establishing the temporospatial and parenchymal pattern of white-matter involvement and in documenting the absence of additional foci in the neuraxis. When the latter is combined with paraclinical data, the accuracy of assessment is excellent. In the future, as more cases of IMDS are diagnosed, neuroimaging will be invaluable in charting the natural history of the disease process and the neuroanatomic and physiologic responses to various therapeutic manipulations.

Original languageEnglish (US)
Pages (from-to)176-180
Number of pages5
JournalAmerican Journal of Neuroradiology
Volume10
Issue number1
StatePublished - 1989

Fingerprint

Diffuse Cerebral Sclerosis of Schilder
Demyelinating Diseases
Neuroimaging
Myelin Sheath
Multiple Sclerosis
Regeneration
White Matter
Therapeutics

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

Inflammatory myelinoclastic diffuse sclerosis (Schilder's disease) : Neuroradiologic findings. / Mehler, Mark F.; Rabinowich, L.

In: American Journal of Neuroradiology, Vol. 10, No. 1, 1989, p. 176-180.

Research output: Contribution to journalArticle

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