title: Brain edema with clasmatodendrosis complicating ataxia telangiectasia.
abstract: Ataxia-telangiectasia is a chronic progressive disorder affecting the nervous and immune systems, caused by a genetic defect in the ATM protein. Clasmatodendrosis, a distinct form of astroglial death, has rarely been reported in ataxia-telangiectasia. Neuropathology of our patient disclosed diffuse edema of the cerebral and cerebellar white matter with prominent clasmatodendrosis, implicating ATM in the regulation of astroglial cell death.
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Japan. Electronic address: konomi-@mbb.nifty.ne.jp.
authors.forename: Konomi
authors.initials: K
authors.lastname: Shimoda
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Japan.
authors.forename: Masakazu
authors.initials: M
authors.lastname: Mimaki
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Japan.
authors.forename: Shuhei
authors.initials: S
authors.lastname: Fujino
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Japan.
authors.forename: Masato
authors.initials: M
authors.lastname: Takeuchi
authors.affiliation: Department of Pathology, Graduate School of Medicine, The University of Tokyo, Japan.
authors.forename: Rumi
authors.initials: R
authors.lastname: Hino
authors.affiliation: Department of Pathology, Graduate School of Medicine, The University of Tokyo, Japan; Department of Pathology, Graduate School of Medicine, The University of Teikyo, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Uozaki
authors.affiliation: Department of Brain Development and Neural Regeneration, Tokyo Metropolitan Institute of Medical Science, Japan.
authors.forename: Masaharu
authors.initials: M
authors.lastname: Hayashi
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Oka
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Japan; Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Mizuguchi
mesh_terms: D000328:Adult; D001253:Astrocytes / Q000473:pathology*; D001260:Ataxia Telangiectasia / Q000150:complications* / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D001921:Brain / Q000473:pathology*; D001929:Brain Edema / Q000150:complications* / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D017809:Fatal Outcome; D006801:Humans; D008297:Male
keywords: Ataxia-telangiectasia; Clasmatodendrosis; NF-kappa B; Tumor suppressor protein p53