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SCHILDER'S DISEASE (ENCEPHALITIS PERIAXIALIS DIFFUSA)

SCHILDER'S DISEASE (ENCEPHALITIS PERIAXIALIS DIFFUSA) 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 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American journal of diseases of children American Medical Association

SCHILDER'S DISEASE (ENCEPHALITIS PERIAXIALIS DIFFUSA)

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Publisher
American Medical Association
Copyright
Copyright © 1930 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
0096-8994
eISSN
1538-3628
DOI
10.1001/archpedi.1930.01930150127015
Publisher site
See Article on Publisher Site

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

Journal

American journal of diseases of childrenAmerican Medical Association

Published: Mar 1, 1930

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