Schilder's myelinoclastic diffuse sclerosis.

We report here a case of Schilder's myelinoclastic diffuse sclerosis in a 14-year-old girl with sudden bilateral visual loss. Computed tomographic scan showed two large symmetrical lesions in the occipital lobes and a smaller hypodense area in the frontal lobe. Cerebrospinal fluid examination revealed increased immunoglobulin G fraction with the presence of oligoclonal bands. Ultrastructural study of a biopsy specimen disclosed a demyelinating disorder with no cytoplasmic inclusions. Steroid treatment was followed by a dramatic response, with almost complete visual recovery and shrinkage of the lesions.