急性播散性脑脊髓炎的研究进展.PDFVIP

中国现代神经疾病杂志 年 月第 卷第 期 ·816 · 2013 9 13 9 ChinJContempNeurolNeurosurg,September2013,Vol.13,No.9 综述 · · 急性播散性脑脊髓炎的研究进展 刘峥 董会卿 【 】 急性播散性脑脊髓炎是一种特发性中枢神经系统炎症性脱髓鞘疾病，可发生于病毒感染 摘要 或疫苗接种后，以儿童多见，成人亦可发生。临床表现为发热、头痛、脑膜刺激征，以及精神异常、癫 发 作、局灶性神经系统症状与体征；临床过程可轻可重，可以为单相型、复发型和多相型。神经影像学检查 可见累及灰质、白质和脊髓的脱髓鞘病灶，双侧均可受累，部分病灶伴强化。诊断标准要求多灶性中枢 神经系统白质脱髓鞘和存在脑病症状。鉴别诊断包括病毒性脑炎、多发性硬化、视神经脊髓炎谱系疾 病、原发性中枢神经系统血管炎等。治疗方法有糖皮质激素、静脉注射丙种球蛋白、免疫抑制剂和血浆 置换疗法等。 【 】 脑脊髓炎； 急性病； 综述 关键词 Acutedisseminatedencephalomyelitis:acomprehensivereview LIUZheng,DONGHui⁃qing Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China Corresponding author: LIU Zheng (Email: lzwcy2003@) 【 】 Abstract Acute disseminated encephalomyelitis (ADEM) is a disease that is characterized by an immune⁃mediated inflammatory reaction and demyelination in the central nervous system, including optic nerve, brain and spinal cord, which is common in children, but also appears in adults. ADEM happens probablywithacausativerelationshiptoviraldiseasesorpriorvaccinations. Itcanalsooccurwithoutany cause. The clinical symptoms of ADEM can begin with fever, headache and meningeal signs, followed by abnormal mental status, seizures and focal neurological signs, such as limb pareses, visual decline and speechdisturbances. Andtheirclinicalcoursesmaybemonophasic,recurrentormultiphasic,mildbutalso very aggressive. Neuroimaging may be characterized bylarge demyelinatinglesionsin the brain involving boththewhiteandthegreymatter,andspinalcord. LesionsinADEMaretypicallylargeglobularlesions, multiple, and asymmetric, partially with diffuse or ring⁃like gadolinium enhancement. The diagnosis of