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

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.