Schilder's Disease: Additional Aspects and a Therapeutic Option*

Abstract

An 8-year-old boy presented with large diffuse myelinoclastic bifrontal and right parietal peripherally enhancing white matter lesions with high intracranial presssure. Intravenous treatment with ACTH and high dose cyclophosphamide (cytoxin) resulted in shrinkage of the lesion, loss of peripheral enhancement and clear clinical improvement. After nine months following the onset of the first episode, an isolated left frontal enhancing lesion appeared along with a new right hemiparesis and speech difficulty. The same therapy again resulted in prompt definitive improvement of the CT scan and reversal of the clinical course. This case was extensively investigated and characterized as the 1912 variety of Schilder''s disease, to be distinguished from post-infectious encephalopathy and other forms of white matter disease such as adrenoleukodystrophy. No elevation of CSF IgG, oligoclonal bands, or characteristic abnormality of T-cell subset distribution were identified. However, the pathological picture most closely resembled that of multiple sclerosis. A striking feature of this child''s clinical history was the relatively rapid clinical improvement with immunosuppression.