治疗的目标是恢复在急性期丧失的神经系统功能;启动预防策略,缓解 TM 并发症症状;适当时,开始治疗,以防止疾病复发。
急性神经功能障碍
排除压迫性病灶、急性感染和其他非 TM 疾病后,TM 相关急性神经功能障碍的一线疗法为静脉注射甲基泼尼松龙,一般每日给药一次,持续 3 至 5 日。[20]Jacob A, Weinshenker BG. An approach to the diagnosis of acute transverse myelitis. Semin Neurol. 2008;28:105-120.http://www.ncbi.nlm.nih.gov/pubmed/18256991?tool=bestpractice.comTM 皮质类固醇无对照试验,但本疗法源自急性多发性硬化 (MS) 发作治疗的外推。对皮质类固醇治疗没有反应,或者开展治疗后症状仍持续发展的严重神经功能障碍患者,可以采用血浆置换急救治疗。[20]Jacob A, Weinshenker BG. An approach to the diagnosis of acute transverse myelitis. Semin Neurol. 2008;28:105-120.http://www.ncbi.nlm.nih.gov/pubmed/18256991?tool=bestpractice.com[65]Weinshenker BG, O'Brien PC, Petterson TM, et al. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol. 1999;46:878-886.http://www.ncbi.nlm.nih.gov/pubmed/10589540?tool=bestpractice.com美国神经病学学会的指南提出,在高剂量皮质类固醇治疗无反应的暴发性中枢神经系统脱髓鞘疾病中,可以考虑使用。[66]Cortese I, Chaudhry V, So YT, et al. Evidence-based guideline update: plasmapheresis in neurologic disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2011;76:294-300.http://www.neurology.org/content/76/3/294.longhttp://www.ncbi.nlm.nih.gov/pubmed/21242498?tool=bestpractice.com推荐这一疗法的试验[65]Weinshenker BG, O'Brien PC, Petterson TM, et al. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol. 1999;46:878-886.http://www.ncbi.nlm.nih.gov/pubmed/10589540?tool=bestpractice.com为 II 类证据,因为受试者包括广泛的中枢神经系统脱髓鞘疾病,包括 TM、MS 和视神经脊髓炎 (NMO)。可以考虑静脉注射免疫球蛋白 (IVIG),但没有随机对照试验评估其有效性。[7]Scott TF, Frohman EM, De Seze J, et al. Evidence-based guideline: clinical evaluation and treatment of transverse myelitis: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2011;77:2128-2134.http://www.neurology.org/content/77/24/2128.longhttp://www.ncbi.nlm.nih.gov/pubmed/22156988?tool=bestpractice.com[20]Jacob A, Weinshenker BG. An approach to the diagnosis of acute transverse myelitis. Semin Neurol. 2008;28:105-120.http://www.ncbi.nlm.nih.gov/pubmed/18256991?tool=bestpractice.com[65]Weinshenker BG, O'Brien PC, Petterson TM, et al. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol. 1999;46:878-886.http://www.ncbi.nlm.nih.gov/pubmed/10589540?tool=bestpractice.com
支持性治疗和急性康复
针对症状的支持性疗法,如呼吸困难、疼痛、尿潴留,如果需要,也可以纳入治疗方案中。
呼吸衰竭:少数颈椎 TM 患者中,病灶累及延髓并可能导致神经性呼吸衰竭。[3]Wingerchuk DM, Hogancamp WF, O'Brien PC, et al. The clinical course of neuromyelitis optica (Devic's syndrome). Neurology. 1999;53:1107-1114.http://www.ncbi.nlm.nih.gov/pubmed/10496275?tool=bestpractice.com密切观察呼吸参数,包括测量的最大呼吸压力和用力肺活量,推荐由熟练的急救护理团队参与治疗颈椎脊髓炎病例。
神经性疼痛:可能是急性或慢性。剧烈疼痛可以用麻醉镇痛药,加巴喷丁或卡马西平治疗。[67]Krishnan C, Kaplin AI, Pardo CA, et al. Demyelinating disorders: update on transverse myelitis. Curr Neurol Neurosci Rep. 2006;6:236-243.http://www.ncbi.nlm.nih.gov/pubmed/16635433?tool=bestpractice.com慢性疼痛通常对抗惊厥药物或三环类抗抑郁药有反应。
急性尿潴留:可采用膀胱导管。残留的神经性膀胱功能障碍症状可能包括急迫性尿失禁、潴留,或混合性障碍,每一种都需要特异性治疗。[67]Krishnan C, Kaplin AI, Pardo CA, et al. Demyelinating disorders: update on transverse myelitis. Curr Neurol Neurosci Rep. 2006;6:236-243.http://www.ncbi.nlm.nih.gov/pubmed/16635433?tool=bestpractice.com
深静脉血栓的预防:不能运动的患者风险会增加。根据通用医疗和骨科手术患者的经验推测,皮下注射肝素或依诺肝素加下肢压力袜或设备可以减少深静脉血栓的风险。
急性康复包括被动和主动的四肢关节活动度维持治疗;减少痉挛状态、痉挛和挛缩风险,减少褥疮性溃疡风险。
对有多发性硬化风险或横断性脊髓炎反复发作风险患者的预防治疗
特发性 TM 患者不需要预防治疗。急性部分性 TM 患者,如果磁共振成像表明典型脱髓鞘病灶有发展为 MS 的风险,可以使用 β 干扰素治疗或格拉默疗法。[35]Jacobs LD, Beck RW, Simon JH, et al. Intramuscular interferon beta-1a therapy initiated during a first demyelinating event in multiple sclerosis. N Engl J Med. 2000;343:898-904.http://www.nejm.org/doi/full/10.1056/NEJM200009283431301#t=articleTophttp://www.ncbi.nlm.nih.gov/pubmed/11006365?tool=bestpractice.com[36]Kappos L, Polman CH, Freedman MS, et al. Treatment with interferon beta-1b delays conversion to clinically definite and McDonald MS in patients with clinically isolated syndromes. Neurology. 2006;67:1242-1249.http://www.ncbi.nlm.nih.gov/pubmed/16914693?tool=bestpractice.com[68]Comi G, Martinelli V, Rodegher M, et al. Effect of glatiramer acetate on conversion to clinically definite multiple sclerosis in patients with clinically isolated syndrome (PreCISe study): a randomised, double-blind, placebo-controlled trial. Lancet. 2009;374:1503-1511.http://www.ncbi.nlm.nih.gov/pubmed/19815268?tool=bestpractice.com
水通道蛋白 -4 自身抗体(视神经脊髓炎 IgG)阳性的患者,建议至少 5 年免疫抑制治疗,因为他们是复发性 TM 和视神经脊髓炎高风险人群。[4]Weinshenker BG, Wingerchuk DM, Vukusic S, et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol. 2006;59:566-569.http://www.ncbi.nlm.nih.gov/pubmed/16453327?tool=bestpractice.com