title: Biopsy-Supported Tumefactive Demyelination of the Central Nervous System in Children.
abstract: Pediatric tumefactive lesions remains challenging to clinicians in terms of diagnosis and treatment. The authors describe 11 children with biopsy-proven central nervous system tumefactive demyelination. The mean age of onset was 11 years. Clinical and radiological data coupled with biopsy aided in the diagnosis of tumefactive demyelination. Of the 6 cases in which oligoclonal band data were available, only 3 showed oligoclonal band in the cerebrospinal fluid. Due to poor recovery despite treatment with high-dose glucocorticosteroids, intravenous immunoglobulin, and/or plasmapheresis, 6 cases went on to receive cyclophosphamide with marked improvement. Long-term data were available on 9 cases. Eight of 9 cases were started on preventative multiple sclerosis therapy after initial presentation; 1 is pending discussion with family. Five of the 8 cases had clinical relapse during treatment. Seven cases met 2010 McDonald criteria for multiple sclerosis at follow-up, (1 developed secondary progressive multiple sclerosis), and 2 cases remained as clinically isolated syndrome on treatment.
authors: Department of Neurology, University of California, San Francisco, San Francisco, CA, USA seem.bh@gmail.com.
authors: Seema Bangalore
authors: SB
authors: Hanumanthe
authors: Department of Neurology, University of California, San Francisco, San Francisco, CA, USA.|Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
authors: Carla
authors: C
authors: Francisco
authors: Department of Neurology, University of California, San Francisco, San Francisco, CA, USA.
authors: Janace
authors: J
authors: Hart
authors: Department of Neurology, University of California, San Francisco, San Francisco, CA, USA.
authors: Jennifer
authors: J
authors: Graves
authors: Department of Neurology, University of California, San Francisco, San Francisco, CA, USA.|Department of Pediatrics, University of California, San Francisco, San Francisco, CA, USA.
authors: Emmanuelle
authors: E
authors: Waubant
mesh_terms: D001706:Biopsy; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D002493:Central Nervous System Diseases / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000473:pathology* / Q000628:therapy; D002648:Child; D003711:Demyelinating Diseases / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000473:pathology* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies
keywords: brain biopsy; children; cyclophosphamide; magnetic resonance imaging; multiple sclerosis; tumefactive demyelination