Brain edema with clasmatodendrosis complicating ataxia telangiectasia.

Brain Dev

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.

Published: August 2017


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Article 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.

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http://dx.doi.org/10.1016/j.braindev.2017.02.007DOI Listing

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