Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Biostatistics Unit, Clinical and Translational Research Center, Keio University Hospital, Tokyo, Japan.
Eur J Neurol. 2024 May;31(5):e16249. doi: 10.1111/ene.16249. Epub 2024 Feb 20.
The aim of this study was to determine the prevalence of anti-myelin-associated glycoprotein (MAG) neuropathy and the current status of such patients in Japan.
We conducted a nationwide survey in 2021 using established epidemiological methods. Questionnaires were sent to all neurology and pediatric neurology departments throughout Japan to identify patients with anti-MAG neuropathy. An initial questionnaire was used to determine the number of patients, with a second one used to collect detailed clinical information.
The estimated number of patients with anti-MAG neuropathy was 353, with a prevalence of 0.28 per 100,000 and an incidence of 0.05 per 100,000. The detailed clinical profiles of 133 patients were available. The median (range) age of onset was 67 (30-87) years, with a prominent peak in the age range 66-70 years, and the male-to-female ratio was 3.6. Most patients had distal sensory-predominant polyneuropathy, and neuropathic pain (50%), or sensory ataxia (42%), while 18% had Waldenström's macroglobulinemia or multiple myeloma. Intravenous immunoglobulin was the most frequently used treatment (65%), but the response rate was <50%, whereas rituximab was given in 32% of patients, and 64% of these showed improvement. At the last visit, 27% of patients could not walk independently.
This study on anti-MAG neuropathy provides updated insights into the epidemiology of this disease, clinical profiles, and treatment approaches in Japan. Rituximab therapy, used for only one-third of the patients, demonstrated efficacy. During the final visit, a quarter of the patients were unable to walk independently. Further studies are warranted to determine the optimal management of this rare and intractable disorder.
本研究旨在确定日本抗髓鞘相关糖蛋白（MAG）神经病的患病率及此类患者的现状。
我们于 2021 年采用既定的流行病学方法进行了一项全国性调查。向日本各地的神经内科和儿科神经内科部门发放问卷，以确定抗 MAG 神经病患者。最初的问卷用于确定患者人数，而第二个问卷则用于收集详细的临床信息。
抗 MAG 神经病患者的估计人数为 353 例，患病率为 0.28/10 万，发病率为 0.05/10 万。共有 133 例患者的详细临床资料可供分析。发病中位（范围）年龄为 67（30-87）岁，66-70 岁年龄组有明显高峰，男女比例为 3.6。大多数患者有以远端感觉为主的多发性神经病，伴有神经痛（50%）或感觉性共济失调（42%），18%患有瓦尔登斯特伦巨球蛋白血症或多发性骨髓瘤。静脉注射免疫球蛋白是最常用的治疗方法（65%），但应答率<50%，而利妥昔单抗在 32%的患者中使用，其中 64%的患者病情改善。在最后一次就诊时，27%的患者无法独立行走。
本项关于抗 MAG 神经病的研究提供了日本该疾病的流行病学、临床特征和治疗方法的最新见解。仅三分之一的患者使用的利妥昔单抗治疗显示出疗效。在最后一次就诊时，四分之一的患者无法独立行走。需要进一步研究以确定这种罕见且难治性疾病的最佳管理方法。
