Kim Se Hee, Millichap John J, Koh Sookyong
Epilepsy Center, Ann & Robert H. Lurie Children's Hospital of Chicago, Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA.
Child Neurol Open. 2016 Apr 4;3:2329048X16633629. doi: 10.1177/2329048X16633629. eCollection 2016 Jan-Dec.
Hemimegalencephaly, a congenital brain malformation typically characterized by enlargement of one hemisphere, is frequently associated with intractable epilepsy. The authors report a case of a 12-month-old girl with hemimegalencephaly who underwent semiurgent hemispherectomy because of rapidly escalating seizures, arrested development, and associated encephalopathy. The brain tissue was examined and evaluated for neuroinflammation. Immunohistochemical analysis of the brain tissue revealed the presence of abundant activated CD68-positive microglia and reactive astrogliosis. Detection of active inflammatory changes in the brain of a patient with hemimegalencephaly complicated by intractable epilepsy suggests a potential role of ongoing brain inflammation in seizure exacerbation and epileptic encephalopathy.
半侧巨脑畸形是一种先天性脑畸形，其典型特征是一侧半球增大，常与难治性癫痫相关。作者报告了一例12个月大患有半侧巨脑畸形的女孩，因其癫痫发作迅速升级、发育停滞和相关脑病而接受了半紧急半球切除术。对脑组织进行了检查并评估了神经炎症。脑组织的免疫组织化学分析显示存在大量活化的CD68阳性小胶质细胞和反应性星形胶质细胞增生。在患有难治性癫痫的半侧巨脑畸形患者的大脑中检测到活跃的炎症变化，提示持续的脑部炎症在癫痫发作加重和癫痫性脑病中可能起作用。
