Schilder's Myelinoclastic Diffuse Sclerosis
1 From the Department of Neurology, Harvard Medical School, and Neurological Unit, Beth Israel Hospital, Boston; and Clinique de Génétique Médicale and INSERM U 12, Hôpital Necker-Enfants Malades, Paris
The term "Schilder's disease" has been used to describe conditions as disparate as adrenoleukodystrophy, myelinoclastic diffuse sclerosis, and postinfectious and postvaccinal encephalomyelitis. The eponymic designation should be reserved for instances of myelinoclastic diffuse sclerosis that correspond to the case described by Schilder in 1912. The diagnosis cannot be made unless adrenoleukodystrophy has been ruled out by analysis of the long-chain fatty acids of plasma cholesterol esters. Schilder's myelinoclastic diffuse sclerosis, a variant of multiple sclerosis, is a very rare disease that occurs in children and adults of both sexes and appears to respond to vigorous treatment with corticosteroids and/or corticotropin. A case of this disease is reported and the recent literature of cases that have been called Schilder's disease is reviewed.
Key Words: Schilder's disease • diffuse sclerosis • myelinoclasia
Submitted on May 8, 1984
Accepted on July 9, 1984
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