Umapathi T, Hughes R A C, Nobile-Orazio E, Léger J M
Department of Neurology, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore, Singapore, 308433.
Cochrane Database Syst Rev. 2005 Jul 20(3):CD003217. doi: 10.1002/14651858.CD003217.pub2.
Multifocal motor neuropathy is a distinct clinical entity characterised by progressive, predominantly distal, asymmetrical limb weakness and minimal sensory abnormality. The diagnostic feature of this condition is the presence of multiple partial motor nerve conduction blocks. Controlled trials have demonstrated the efficacy of regular intravenous immunoglobulin infusions. Immunosuppressive agents have been used as primary, second-line or adjunctive agents for its treatment. This review was undertaken to identify and review systematically randomised controlled trials of immunosuppressive agents. The use of intravenous immunoglobulin will be the subject of a separate review.
To provide the best available evidence from randomised controlled trials on the role of immunosuppressive agents for the treatment of multifocal motor neuropathy.
We searched the Cochrane Neuromuscular Disease Group trials register for all trials of multifocal motor neuropathy published, using 'multifocal motor neuropathy' OR 'chronic inflammatory demyelinating polyradiculoneuropathy' OR ' conduction block' OR ' motor neuropathy' AND 'immunosuppressive agents', 'immunosuppressants', 'corticosteroids', 'plasma exchange', 'azathioprine', 'cyclophosphamide', 'cyclosporin', 'ciclosporin', 'methotrexate', and 'mycophenolate', 'immunomodulatory agents', 'interferon', 'total lymphoid irradiation' or 'bone marrow transplantation' as search terms. In addition we searched MEDLINE, EMBASE for 2000 and 2001 and CINAHL, LILACS for all years. We updated the register search in February 2004 and searched MEDLINE (January 1966 to end May 2004) and EMBASE (January 1980 to end May 2004).
All randomised controlled trials and quasi-randomised clinical trials in which allocation was not random but was intended to be unbiased (e.g. alternate allocation) were to have been selected. Since no such trials were discovered, all prospective and retrospective case series were included in the 'background' or 'discussion' sections of the review.
All studies on multifocal motor neuropathy or lower motor neuron weakness with conduction block and no sensory abnormality were scrutinised for data on patients treated with any form of immunosuppressive agents besides intravenous immunoglobulin. The information on the outcome of treatment was then collated and summarised.
We found no randomised controlled trials of any immunosuppressive agents for multifocal motor neuropathy. We summarised the results of retrospective and prospective case series in the discussion of the review.
AUTHORS' CONCLUSIONS: There are no randomised controlled trials to indicate whether immunosuppressive agents are beneficial in multifocal motor neuropathy.
多灶性运动神经病是一种独特的临床病症，其特征为进行性、主要为远端、不对称的肢体无力以及轻微的感觉异常。该病症的诊断特征是存在多个部分运动神经传导阻滞。对照试验已证明定期静脉注射免疫球蛋白的疗效。免疫抑制剂已被用作其治疗的一线、二线或辅助药物。本综述旨在系统地识别和回顾免疫抑制剂的随机对照试验。静脉注射免疫球蛋白的使用将另行综述。
提供来自随机对照试验的最佳现有证据，以证明免疫抑制剂在多灶性运动神经病治疗中的作用。
我们在Cochrane神经肌肉疾病组试验注册库中搜索了所有已发表的多灶性运动神经病试验，使用“多灶性运动神经病”或“慢性炎症性脱髓鞘性多发性神经根神经病”或“传导阻滞”或“运动神经病”以及“免疫抑制剂”、“免疫抑制药”、“皮质类固醇”、“血浆置换”、“硫唑嘌呤”、“环磷酰胺”、“环孢素”、“环孢菌素”、“甲氨蝶呤”和“霉酚酸酯”、“免疫调节药”、“干扰素”、“全身淋巴照射”或“骨髓移植”作为检索词。此外，我们搜索了MEDLINE、2000年和2001年的EMBASE以及所有年份的CINAHL、LILACS。我们于2004年2月更新了注册库搜索，并搜索了MEDLINE（1966年1月至2004年5月底）和EMBASE（1980年1月至2004年5月底）。
所有随机对照试验和准随机临床试验，其中分配并非随机但旨在无偏倚（例如交替分配）均应被选中。由于未发现此类试验，所有前瞻性和回顾性病例系列均纳入综述的“背景”或“讨论”部分。
对所有关于多灶性运动神经病或伴有传导阻滞且无感觉异常的下运动神经元无力的研究进行审查，以获取除静脉注射免疫球蛋白外接受任何形式免疫抑制剂治疗患者的数据。然后整理并总结治疗结果信息。
我们未发现任何关于免疫抑制剂治疗多灶性运动神经病的随机对照试验。我们在综述的讨论部分总结了回顾性和前瞻性病例系列的结果。
尚无随机对照试验表明免疫抑制剂对多灶性运动神经病是否有益。
