Brain edema with clasmatodendrosis complicating ataxia telangiectasia

作者：Shimoda Konomi^*; Mimaki Masakazu; Fujino Shuhei; Takeuchi Masato; Hino Rumi; Uozaki Hiroshi; Hayashi Masaharu; Oka Akira; Mizuguchi Masashi

来源：Brain & Development, 2017, 39(7): 629-632.

DOI：10.1016/j.braindev.2017.02.007

• Ataxia-telangiectasia
• Clasmatodendrosis
• NF-kappa B
• Tumor suppressor protein p53

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.

• 出版日期2017-8
• 单位河北医科大学