Myelinoclastic diffuse sclerosis (Schilder's disease): cliniconeuroradiologic correlations

We report a 17-year-old boy with myelinoclastic diffuse sclerosis (Schilder's disease) presenting with left leg paresis, visual loss, and behavioral changes. CT and MRI showed two large lesions in the subcortical white matter of the occipital and parietal lobes of both hemispheres and increased intracranial pressure. Histology disclosed large areas of demyelination and perivascular infiltrates. The patient improved with coincident oral prednisolone treatment.