Muscle Nerve. 2014 Mar;49(3):357-61. doi: 10.1002/mus.23930.
Our objective was to do an epidemiologic survey of patients with multifocal motor neuropathy (MMN) in comparison with those with amyotrophic lateral sclerosis (ALS) in Japan.
In this retrospective study, we examined 46 patients with MMN and 1,051 patients with ALS from major neuromuscular centers in Japan from 2005 to 2009. Diagnosis was based on the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) and the revised El Escorial criteria. The efficacy of intravenous immunoglobulin (IVIg) was also taken into consideration in the diagnosis of MMN.
The ratio of MMN to ALS patients (0–0.10) varied among the centers, but mostly converged to 0.05. The prevalence was estimated to be 0.29 MMN patients and 6.63 ALS patients per 100,000 population.
The frequency of MMN patients was around 1 out of 20 ALS patients, and MMN was possibly underdiagnosed in some centers.
本研究旨在对日本的多发性运动神经病(MMN)患者与肌萎缩侧索硬化症(ALS)患者进行流行病学调查。
本回顾性研究纳入了 2005 年至 2009 年期间日本主要神经肌肉中心的 46 例 MMN 患者和 1051 例 ALS 患者。诊断标准基于欧洲神经病学学会/周围神经病学会(EFNS/PNS)和修订后的 El Escorial 标准。在 MMN 的诊断中也考虑了静脉注射免疫球蛋白(IVIg)的疗效。
各中心 MMN 与 ALS 患者的比例(0-0.10)存在差异,但大多集中在 0.05。估计 MMN 患者的患病率为每 100,000 人口中有 0.29 例,ALS 患者的患病率为 6.63 例。
MMN 患者的频率约为每 20 例 ALS 患者中就有 1 例,且在某些中心 MMN 可能被漏诊。