Rajabally Yusuf A, Englezou Christina, Cluett Grace, Bellanti Roberto, Rinaldi Simon, Chin Mow Wei, Hadden Robert, Roman Madalina, Hewamadduma Channa, Murray Ashleigh, Elsaddig Amar, Lavin Tim, Cousins Oliver, Nirmalananthan Niranjanan, Freiha Joumana, Osman Chinar, Evans Matthew, Carr Aisling, Holt James K L
Aston Medical School, Aston University, Birmingham, UK.
Inflammatory Neuropathy Clinic, Department of Neurology, University Hospitals Birmingham, Birmingham, UK.
J Peripher Nerv Syst. 2025 Jun;30(2):e70018. doi: 10.1111/jns.70018.
We conducted a survey to determine the current diagnosis and treatment of multifocal motor neuropathy (MMN) in the United Kingdom.
Demographic, diagnostic and treatment data were collected at nine UK neuroscience centres.
Ninety-five subjects were included. Mean age at diagnosis was 49.9 years (SD: 11.4). Males were more commonly affected (ratio: 1.9:1). Diagnostic delay was > 1 year from the time of first neurological assessment, in > 50% of subjects. Applying modified EFNS/PNS 2010 criteria, 69/95 (72.6%) had definite MMN, 10/95 (10.5%) had probable MMN, 15/95 (15.8%) had possible MMN, through treatment responsiveness in 9/15 (60%) and 1/95 (1.1%) did not meet criteria. Cerebrospinal fluid examination, anti-GM1 antibody testing and brachial plexus magnetic resonance imaging were non-contributory. Immunoglobulin response was reported in 90/92 subjects (97.8%), and 84/90 (93.3%) remained on treatment after a mean of 9.4 years, at a mean dose of 26.2 g/week (range: 4-114). Mean long-term immunoglobulin dose was 30%-60% higher than reported in neighbouring countries. Contrasting with previous reports of frequent loss of immunoglobulin response and functional decline, our physician-assessed long-term outcome was favourable (stable or improving) in 74/84 (88.1%) treated subjects.
MMN diagnosis and treatment in the United Kingdom are comparable to that of neighbouring countries and follow existing guidelines. Diagnostic delay after the first neurological assessment is considerable. Electrophysiology shows at least one definite/probable conduction block in nearly 90% of cases. The mean long-term immunoglobulin dose is higher in the United Kingdom than reported elsewhere, although highly variable. Whether higher doses of immunoglobulin may improve long-term outcomes requires further study.
我们开展了一项调查，以确定英国多灶性运动神经病（MMN）的当前诊断和治疗情况。
在英国的九个神经科学中心收集了人口统计学、诊断和治疗数据。
纳入了95名受试者。诊断时的平均年龄为49.9岁（标准差：11.4）。男性受影响更为常见（比例：1.9:1）。超过50%的受试者从首次神经学评估起诊断延迟超过1年。应用修改后的EFNS/PNS 2010标准，95例中有69例（72.6%）确诊为MMN，10例（10.5%）可能为MMN，15例（15.8%）可能为MMN，其中9例（60%）通过治疗反应确诊，1例（1.1%）不符合标准。脑脊液检查、抗GM1抗体检测和臂丛磁共振成像均无诊断价值。92例受试者中有90例（97.8%）报告有免疫球蛋白反应，90例中的84例（93.3%）在平均9.4年后仍在接受治疗，平均剂量为26.2克/周（范围：4 - 114）。英国的平均长期免疫球蛋白剂量比邻国报告的高30% - 60%。与先前关于免疫球蛋白反应频繁丧失和功能衰退的报告形成对比，我们经医生评估的长期结果显示，84例接受治疗的受试者中有74例（88.1%）情况良好（稳定或改善）。
英国的MMN诊断和治疗与邻国相当，且遵循现有指南。首次神经学评估后的诊断延迟相当长。电生理学显示近90%的病例至少有一处明确/可能的传导阻滞。英国的平均长期免疫球蛋白剂量高于其他地方报告的剂量，尽管差异很大。更高剂量的免疫球蛋白是否能改善长期结果需要进一步研究。
