摘要
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。
Acute disseminate d encephalomyelitis(ADEM) is a disease that is characterize d by an immune me diate d inflammatory reaction an d demyelination in the central nervous system, inclu ding optic nerve, brain an d spinal cor d, which is common in chil dren, but also appears in a dults. ADEM happens probably with a causative relationship to viral diseases or prior vaccinations. It can also occur without any cause. The clinical symptoms of ADEM can begin with fever, hea dache an d meningeal signs, followe d by abnormal mental status, seizures an d focal neurological signs, such as limb pareses, visual decline an d speech disturbances. An d their clinical courses may be monophasic, recurrent or multiphasic, mil d but also very aggressive. Neuroimaging may be characterize d by large demyelinating lesions in the brain involving both the white an d the grey matter, an d spinal cor d. Lesions in ADEM are typically large globular lesions,multiple, an d asymmetric, partially with diffuse or ring like ga dolinium enhancement. The diagnosis of ADEM requires both multifocal involvement an d encephalopathy by consensus criteria. The differential diagnoses of ADEM inclu de a variety of disor ders, such as viral encephalitis, multiple sclerosis,neuromyelitis optica spectrum disor ders an d primary central nervous system vasculitis, etc. Treatments of ADEM inclu de corticosteroi ds, intravenous immunoglobulin, plasmapheresis an d other immunosuppressive agents.
出处
《中国现代神经疾病杂志》
CAS
2013年第9期816-820,共5页
Chinese Journal of Contemporary Neurology and Neurosurgery
基金
国家自然科学基金资助项目(项目编号:30800351)
北京市科技新星计划项目(项目编号:2008B73)~~
关键词
脑脊髓炎
急性病
综述
Encephalomyelitis
Acute disease
Review