Pediatric case of the day. Myelinoclastic diffuse sclerosis (MDS) (Schilder disease).

Bonsack, T A; Robertson, R; Lacson, Atilano; Casadonte, Joseph; Buonomo, Carlo

doi:10.1148/radiographics.16.6.8946552

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1998

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“…Many of these studies, however, were published before reliable biochemical and strict clinical criteria were developed for the diagnosis of this disorder and, thus, suffer from contamination of other disease entities [4]. A few papers discussing the presentation and management of Schilder's disease do appear in the recent literature, but discussion of the variable presentation and longterm follow-up is sparse [5].…”

Section: Introductionmentioning

confidence: 99%

Presentation, Management and Follow-Up of Schilder’s Disease

Garell

Menezes²,

Baumbach³

et al. 1998

Pediatr Neurosurg

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Schilder’s diffuse myelinoclastic sclerosis is a rare demyelinating disease which often mimics intracranial neoplasm or abscess. We have treated 3 patients with this disorder in the past 5 years and followed their postoperative course. Certain distinct features of this disease will allow neurosurgeons to preoperatively entertain this diagnosis. We discuss postoperative treatment and propose a new hypothesis regarding the variable prognoses of this disorder. Schilder’s disease constitutes an important diagnosis for any neurosurgeon to be aware of (especially those treating the pediatric age group) which has not received adequate coverage in the neurosurgical literature.

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Section: Introductionmentioning

confidence: 99%