Schilder's disease in a young child with tumefactive demyelinating brain lesion

Abstract

Schilder’s disease is a rare sporadic demyelinating disease of the brain. We report a girl with Schilder’s disease who had undergone Kasai operation for biliary atresia. The patient presented with acute right hemiparesis. Brain magnetic resonance imaging (MRI) showed a single large tumefactive white matter lesion. A diagnosis of Schilder’s disease was based on clinical features and MRI findings. The patient showed dramatic clinical improvement and significant regression of the lesion in response to high-dose intravenous methyl prednisone, and remained free of relapse of other neurologic problems during the 3-year follow-up