Walsh & Hoyt: Myelinoclastic Diffuse Sclerosis (Encephalitis Periaxialis Diffusa; Schilder's Disease)

In 1912, Schilder reported the case of a 12-year-old girl who experienced rapidly progressive mental deterioration associated with signs of increased ICP and death within 19 weeks. Postmortem examination disclosed large, well-demarcated areas of demyelination in the white matter of both cerebral hemispheres and a number of smaller demyelinating foci that resembled the typical plaques of MS. There was a prominent inflammatory reaction in both types of lesions with relative sparing of axon cylinders. Because of the similarities of the pathologic changes in this case to those of MS, Schilder called this disease ""encephalitis periaxalis diffusa"" to differentiate it from ""encephalitis periaxalis scleroticans,"" the term that had previously been used by Marburg to describe a case of acute MS. Unfortunately, Schilder subsequently used the same term for two other completely different conditions. One seems to have been a case of adrenoleukodystrophy and the other a case of subacute sclerosing panencephalitis. These later reports confused the subject for many years, and cases of adrenoleukodystrophy are still often called ""Schilders disease."" Nevertheless, if one eliminates the hereditary metabolic dystrophies and the various childhood disorders of cerebral white matter that are called ""Schilders disease,"" there remains a characteristic group of cases that does correspond to Schilders original description. These latter cases, which are often called myelinoclastic diffuse sclerosis, are nonfamilial, do not follow an obviously viral exanthem, and are not characterized pathologically by inclusion bodies or viral particles in the CNS. Throughout the remainder of this section, we refer to this condition as ""myelinoclastic diffuse sclerosis.""