青少年起病的急性坏死性脑病的临床特点(附1例报告)  

Clinical features of adolescent-onset acute necrotizing encephalopathy(report of one case)

作　　者：丁俭 刘猛[1] 李达文 盛世英[1] 岳春贤 DING Jian;LIU Meng;LI Da-wen(Department of Neurology,the Third Affiliated Hospital of Soochow University(the First People’s Hospital of Changzhou),Changzhou 213003,China)

机构地区：[1]苏州大学附属第三医院(常州市第一人民医院)神经内科,常州213003

出　　处：《临床神经病学杂志》2023年第2期130-133,共4页Journal of Clinical Neurology

基　　金：常州市科技计划资助项目(CJ20220201)。

摘　　要：目的探讨青少年起病的急性坏死性脑病(ANE)临床特点,以提高对该疾病的认识。方法报道一例青少年急性坏死性脑病患者的临床资料,进行诊断、鉴别诊断和预后分析,并对相关文献进行复习。结果患者为17岁女性,急性起病,迅速进展,主要表现为头痛、意识障碍和癫痫发作,CSF细胞学和生化指标均正常,头颅MRI检查示双侧基底节、丘脑、脑干对称性受累。给予糖皮质激素、免疫球蛋白等治疗仍进行性加重,自动出院后死亡。结论ANE是一种快速进展性脑病,病死率高,预后差,早期识别和及时治疗至关重要。ANE影像学表现具有一定特征性,有助于早期诊断。Objective To explore the clinical features of adolescent-onset acute necrotizing encephalopathy(ANE)for better understanding of this disease.Methods Diagnosis,differential diagnosis and prognosiswere analyzed according to the clinical data of one case of ANE.Relevant literatures were reviewed.Results The patient was a 17-year-old adolescent with acute onset and presented with headache,seizures and disturbance of consciousness.The imaging changes of ANE showed symmetric multifocal brain lesions,especially bilateral thalamic abnormal signals.Although steroidsand intravenous immunoglobulin were applied,the patient’s condition still deteriorated quickly.Finally the patient died after leaving hospital voluntarily.Conclusions ANE is a rapid progressive encephalopathy with high mortality rate and poorprognosis.Early identification and timely intervention are critically important.The typical imaging features on MRI are helpful for early diagnosis.

关 键 词：急性坏死性脑病 青少年 影像学特征 预后 

分 类 号：R742[医药卫生—神经病学与精神病学]