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

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. Neurology Asia 2013; 18(4) : 419 – 421 Address correspondence to: Hoon-Chul Kang, M.D., Ph.D., Division of Pediatric Neurology, Department of Pediatrics, Pediatric Epilepsy Clinics, Severance Children’s Hospital, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea. Tel: 82-2-2228-2075, Fax: 82-2-393-9118, E-mail: [email protected] INTRODUCTION Schilder’s disease, also known as myelinoclastic diffuse sclerosis, was first described by Schilder in 1912. It is a sporadic subacute demyelinating brain disease that usually affects children. This rare disease results in formation of one or two bilateral large tumefactive plaques. The clinical presentation is atypical for pediatric multiple sclerosis, and radiologic findings often mimic brain tumor or abscess. Clinical course or treatments for Schilder’s disease have not been clearly established. However, some radiological characteristics and clinical features suggest differential diagnosis from other demyelinating diseases, brain tumors, or abscess. Here we contribute to the understanding of Schilder’s disease by presenting the case of a child with Schilder’s disease who underwent a Kasai operation for biliary atresia.