摘要该例急性坏死性脑病(ANE)患者青年起病,发病前有上呼吸道感染病史,主要表现为癫痫发作、意识障碍,头颅MRI检查示双侧丘脑区、脑桥被盖、小脑半球对称性受累.影像学改变与病理生理改变相对应,起始表现为脑肿胀、水肿,急性期可见受累脑组织出血、坏死,恢复期表现为含铁血黄素沉积、囊腔形成,符合ANE诊断.早期使用大剂量丙种球蛋白和甲泼尼龙冲击治疗,患者预后良好,证明激素、丙种球蛋白免疫抑制治疗对ANE有效.

abstractsA young female patient with acute necrotizing encephalopathy (ANE) is reported,who aged 15 years,with a history of upper respiratory tract infection,main clinical manifestations of seizures and consciousness disorders,and brain MRI examination showing characteristic symmetrical bilateral abnormal signals at both thalamic area,pons,and cerebellar hemisphere.Imaging changes corresponded to pathophysiological changes.The initial manifestations were found to be brain swelling and edema.In the acute phase,hemorrhage and necrosis of the affected brain tissues were observed.The recovery period was characterized by hemosiderin deposition and cystic space formation,which was consistent with ANE diagnosis.By early use of high-dose gammaglobulin and methylprednisolone,the prognosis of the patient was good,proving that immunosuppressive therapy by corticosteroids and gammaglobulin is effective for ANE.