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Sex-linked sudanophilic leukodystrophy with adrenocortical atrophy (so-called Schilder's disease)

Report of a case and review of the literature

From the Department of Pathology, The Danbury Hospital, Danbury, CT 06810

We report the clinical and pathologic features of a case of sex-linked sudanophilic leukodystrophy in an 8-year-old boy. Our survey of the pertinent literature on this disease supports the current view that it is not limited to the central nervous system, but is a generalized process, probably caused by an unknown metabolic defect, with a definitive genetic pattern and varying expressions. Although the disease was originally described by Schilder as a single entity, cases like ours show that the term "sudanophilic leukodystrophy" represents a better classification than the usually misinterpreted eponym, "encephalitis periaxialis diffusa."

Dr. Sanchez' address is Department of Pathology, University of Maryland, 22 South Greene Street, Baltimore, Maryland 21201.

Received for publication May 29, 1975.

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