Unit of Neurology and Neuromuscular Diseases, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
UOC Neurologia e Malattie Neuromuscolari, Policlinico G. Martino, via Consolare Valeria 1, 98124, Messina, ME, Italy.
Sci Rep. 2021 Apr 28;11(1):9216. doi: 10.1038/s41598-021-88711-9.
Multifocal motor neuropathy (MMN) is a rare disease with a prevalence of less than 1 per 100,000 people. Intravenous immunoglobulin (IVIG) therapy, performed for a long-term period, has been demonstrated able to improve the clinical picture of MMN patients, ameliorating motor symptoms and/or preventing disease progression. Treatment with subcutaneous immunoglobulin (SCIg) has been shown to be as effective as IVIG. However, previously published data showed that follow-up of MMN patients in treatment with SCIg lasted no more than 56 months. We report herein the results of a long-term SCIg treatment follow up (up to 96 months) in a group of 8 MMN patients (6 M; 2F), previously stabilized with IVIG therapy. Clinical follow-up included the administration of Medical Research Council (MRC) sum-score, the Overall Neuropathy Limitation Scale (ONLS) and the Life Quality Index questionnaire (LQI) at baseline and then every 6 months. Once converted to SCIg, patients' responsiveness was quite good. Strength and motor functions remained stable or even improved during this long-term follow-up with benefits on walking capability, resistance to physical efforts and ability in hand fine movements.
多灶性运动神经病(MMN)是一种罕见疾病,患病率低于每 10 万人中有 1 人。长期静脉注射免疫球蛋白(IVIG)治疗已被证明能够改善 MMN 患者的临床症状,改善运动症状和/或防止疾病进展。皮下免疫球蛋白(SCIg)治疗已被证明与 IVIG 一样有效。然而,之前发表的数据显示,接受 SCIg 治疗的 MMN 患者的随访时间不超过 56 个月。我们在此报告了一组 8 例 MMN 患者(6 例男性;2 例女性)接受长期 SCIg 治疗(最长 96 个月)的结果,这些患者之前已经通过 IVIG 治疗稳定。临床随访包括在基线时和之后每 6 个月进行肌力量研究联合会(MRC)总评分、总体神经功能障碍量表(ONLS)和生活质量指数问卷(LQI)。一旦转换为 SCIg,患者的反应相当好。在长期随访期间,力量和运动功能保持稳定甚至改善,从而提高了行走能力、抵抗体力消耗的能力和手部精细运动的能力。