Department of Neurology, Children's Hospital of Eastern Ontario, University of Ottawa, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada,
Curr Treat Options Neurol. 2012 Jun;14(3):264-75. doi: 10.1007/s11940-012-0170-0.
Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease, characterized by an acute onset of polyfocal central nervous system (CNS) deficits, including encephalopathy, demonstrating multifocal lesions on MRI. ADEM is typically a monophasic disorder, but recurrent and multiphasic courses have been described. Furthermore, an ADEM presentation has been reported in neuromyelitis optica (NMO) and multiple sclerosis (MS), particularly in younger children. CNS infections, other autoimmune diseases, and neurometabolic disorders may mimic ADEM at manifestation. There is no single test confirming the diagnosis of ADEM, and diagnosis is based upon a combination of clinical and radiologic features and exclusion of diseases that resemble ADEM. Therefore, a broad workup including infectious, immunologic, and metabolic tests, as well as a systematic follow-up including MRI, is indicated to establish an accurate diagnosis as a prerequisite for an optimized treatment approach. There is a lack of evidence-based, prospective clinical trial data for the management of ADEM. Empiric antibacterial and antiviral treatment is standard of care until an infectious disease process is ruled out. Based on the presumed autoimmune etiology of ADEM, the common treatment approach consists of intravenous methylprednisolone at a dosage of 20 to 30 mg/kg per day (maximum 1 g/day) for 3 to 5 days, followed by an oral corticosteroid taper of 4 to 6 weeks. In case of insufficient response or contraindications to corticosteroids, intravenous immunoglobulin G (IVIG) at a dosage of 2 g/kg divided over 2 to 5 days is a therapeutic option. For severe or life-threatening cases of ADEM, plasmapheresis should be considered early in the disease course. Decompressive craniectomy has been reported as a life-saving measure for ADEM patients with intracranial hypertension. There is a lack of specific recommendations for the long-term management of recurrent and multiphasic ADEM. In children with relapsing demyelinating events, the diagnosis of a chronic autoimmune CNS disease like MS or NMO should be considered.
急性播散性脑脊髓炎（ADEM）是一种炎症性脱髓鞘疾病，其特征为急性多灶性中枢神经系统（CNS）功能障碍，包括脑病，MRI 显示多灶性病变。ADEM 通常为单相性疾病，但也有复发和多相性病程的报道。此外，视神经脊髓炎（NMO）和多发性硬化症（MS）也有 ADEM 表现，特别是在年幼的儿童中。中枢神经系统感染、其他自身免疫性疾病和神经代谢性疾病在表现上可能与 ADEM 相似。没有单一的检查可以确诊 ADEM，诊断基于临床和影像学特征的综合，并排除与 ADEM 相似的疾病。因此，广泛的检查包括感染、免疫和代谢检查，以及包括 MRI 的系统随访，以明确诊断，这是优化治疗方法的前提。ADEM 的管理缺乏基于证据的前瞻性临床试验数据。在排除感染性疾病过程之前，经验性的抗菌和抗病毒治疗是标准的治疗方法。基于 ADEM 的推测自身免疫病因，常见的治疗方法包括每天 20 至 30 毫克/公斤的静脉甲基强的松龙（最大剂量为 1 克/天），持续 3 至 5 天，然后口服皮质类固醇逐渐减量 4 至 6 周。如果对皮质类固醇治疗反应不足或有禁忌症，则可选择静脉注射免疫球蛋白 G（IVIG），剂量为 2 克/公斤，分 2 至 5 天给予。对于严重或威胁生命的 ADEM 病例，应在疾病早期考虑进行血浆置换。对于有颅内压增高的 ADEM 患者，已报道去骨瓣减压术是一种挽救生命的措施。对于复发性和多相性 ADEM，尚无具体的长期管理建议。对于有复发脱髓鞘事件的儿童，应考虑诊断为 MS 或 NMO 等慢性自身免疫性中枢神经系统疾病。
