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

Controversy has surrounded the rare clinicopathologic entity of inflammatory myelinoclastic diffuse sclerosis (IMDS) since the seminal description of Schilder in 1912 [1]. Since that time several distinct myelinoclastic and dysmyelinating conditions have been grouped under this eponym. To this newly described demyelinating disease, the author planted the seeds of an enduring controversy by including two additional cases (1913, 1924) [2, 3] that, in retrospect, were shown to be examples of a leukodystrophy and, possibly, subacute sclerosing panencephalitis, respectively. As a result, the evolving eponym "Schilder's" has come to represent a much wider spectrum of disease than the original 1912 description, including examples of adrenoleukodystrophy, acute disseminated encephalomyelitis, transitional sclerosis, and juvenile multiple sclerosis. In the context of recent advances in biochemistry, neuroimaging, and electrophysiology, it has been possible to more systematically separate related disorders of white matter. This diagnostic reappraisal has allowed the emergence of a rare IMDS that remains faithful to Schilder's original 1912 description. We present only the third fully documented case. The temporal profile spans the entire spectrum of clinical forms of IMDS previously defined by Lhermitte [4] . Because of the rapid and dramatic fluctuations in the patient's functional status, it has been possible to obtain sequential CT and comparative MR scans that graphically document the interplay of cavitary and diffuse whitematter lesions. This has allowed cliniconeuroradiologic assessment of this rare but distinctive inflammatory myelinoclastic condition, rigorously defined by biochemical, pathologic, and electrophysiologic criteria.