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' neurologic 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 positive history of contact with adult tuberculosis in two of our patients raise the possibility of a connection between tuberculosis and Schilder's disease.

Raised ICP in a child with cryptococcal meningitis: CT evidence of a distal CSF block.

Raised intracranial pressure (ICP) often complicates the course of cryptococcal meningitis. The pathogenesis of the severely raised cerebrospinal fluid (CSF) pressure commonly associated with this condition is largely unexplained, because the majority of patients have normal cranial computed tomographic (CT) findings when diagnosed. We report a case of cryptococcal meningitis in a child who had severely raised CSF pressure on admission, and in whom repeated CT scanning showed progressive enlargement of the subarachnoid space and ventricular system during the course of treatment. The normalization of these spaces after ventriculoperitoneal (VP) shunting suggests a distal CSF block as the cause of the raised ICP in this patient. The CSF pressure was monitored and treatment with oral acetazolamide and furosemide resulted in a definite, but slow and incomplete lowering of ICP. Intrathecal therapy with hyaluronidase had no beneficial effect on either ICP or the degree of visual loss.