SCHILDER'S DISEASE (ENCEPHALITIS PERIAXIALIS DIFFUSA)

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