Original Article Demyelinating Disease of Schilder Type in Three Young South African Children: Dramatic Response to Corticosteroids

Three young children with the Schilder variant of multiple sclerosis were seen within a 3-year period at our hospital. The diagnosis was made on the basis of the typical (but not pathognomonic) clinical and magnetic resonance imaging (MRI) findings after eliminating other demyelinating and post-infectious disorders of the central nervous system. All three patients were treated with prednisone (2 mg/kg/day), which resulted in complete recovery in one patient and mild and moderate residual hemiparesis in the two other patients, respectively. Corticosteroid therapy was continued until the patients ’ neu-rologic condition normalized or no further clinical improvement occurred. No relapses were seen after discontinuation of corticosteroid treatment. Computed tomographic (CT) scan and MRI findings after completion of corticosteroid therapy were equally dramatic and corresponded with the clinical improvement. A strongly positive tuberculin skin test and a pos-itive history of contact with adult tuberculosis in two of our patients raise the possibility of a connection between tuberculosis and Schilder’s disease. (J Child Neurol 1998;13:197-201). In 1912, Schilderl described large, clearcut areas of demyeli-nation in both cerebral hemispheres of a 14-year-old girl who died after a progressive neurologic illness that lasted 19 weeks. Schilder regarded this condition as a juvenile vari