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。

关键词: 脑脊髓炎, 急性病, 综述

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 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, headache and meningeal signs, followed by abnormal mental status, seizures and focal neurological signs, such as limb pareses, visual decline and speech disturbances. And their clinical courses may be monophasic, recurrent or multiphasic, mild but also very aggressive. Neuroimaging may be characterized by large demyelinating lesions in the brain involving both the white and the grey matter, and spinal cord. Lesions in ADEM are typically large globular lesions, multiple, and asymmetric, partially with diffuse or ring-like gadolinium enhancement. The diagnosis of ADEM requires both multifocal involvement and encephalopathy by consensus criteria. The differential diagnoses of ADEM include a variety of disorders, such as viral encephalitis, multiple sclerosis, neuromyelitis optica spectrum disorders and primary central nervous system vasculitis, etc. Treatments of ADEM include corticosteroids, intravenous immunoglobulin, plasmapheresis and other immunosuppressive agents.

Key words: Encephalomyelitis, Acute disease, Review