SCHILDER'S DISEASE (ENCEPHALITIS PERIAXIALIS DIFFUSA)

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Abstract
The term diffuse cerebral sclerosis was long applied to a group of diverse diseases characterized by diffuse glial hyperplasia, admittedly the end-result of varied pathologic conditions including inflammatory, degenerative and developmental lesions. In 1912, Schilder1 isolated from this broad group what he termed a new disease entity, encephalitis periaxialis diffusa, or as it is more commonly termed, Schilder's disease. His original case was that of a child who presented a confusing clinical picture and died after operation for a presumed cerebral tumor. On the basis of this case and of four other cases that he chose from the literature (those of Ceni,2 Rossolimo,3 Beneke,4 and Haberfield and Spieler5), Schilder characterized the new pathologic entity as a sharply delimited but diffuse subcortical symmetrical involvement of the white matter. The myelin sheaths were destroyed with relative preservation of axones, and secondary hyperplasia of the fibrillary glia took

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