Department of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht100, 3584 CX Utrecht, the Netherlands.
Neurology. 2010 Aug 31;75(9):818-25. doi: 10.1212/WNL.0b013e3181f0738e.
Identification and examination of all patients with multifocal motor neuropathy (MMN) in the Netherlands to document the clinical spectrum and response to IV immunoglobulin (IVIg) and to determine correlates of outcome.
A national cross-sectional descriptive study was performed. Ninety-seven patients were identified; 88 participated. Logistic regression analysis was used to study determinants of outcome.
Age at onset was younger in men than in women (38 vs 45 years, p = 0.05). Onset of weakness was in distal arm (61%) or distal leg (34%), and occasionally in the upper arm (5%). Initial diagnosis was motor neuron disease in one-third of patients. Brisk, but not pathologic, reflexes in weakened muscles were found in 8%. Conduction blocks were most frequently detected in the ulnar (80%) and median (77%) nerves, but occasionally only between Erb and axilla (6%), or in the musculocutaneous nerve (1%). Ninety-four percent responded to IVIg therapy: nonresponders had longer disease duration before the first treatment (p = 0.03). Seventy-six percent received IVIg maintenance treatment at the time of this study (median duration 6 years; range 0-17): the median dose increased over the years from 12 to 17 g per week (p < 0.01). Independent determinants of more severe weakness and disability were axon loss (p < 0.001; p < 0.0001) and longer disease duration without IVIg (p = 0.03; p = 0.07).
The results of this study may help aid recognition the clinical picture of MMN. Early IVIg treatment may help to postpone axonal degeneration and permanent deficits.
This study provides Class IV evidence that IVIg improves muscle strength of patients with MMN and disability (defined as an increase of >or=1 Medical Research Council grade in at least 2 muscle groups without decrease in other muscle groups) in 94% (95% confidence interval, 86.8%-97.4%) of patients.
在荷兰识别和检查所有多灶性运动神经病(MMN)患者,记录其临床谱并评估免疫球蛋白静脉滴注(IVIg)的疗效,确定与结局相关的因素。
进行了一项全国性的横断面描述性研究。共发现 97 例患者,88 例参与研究。采用逻辑回归分析研究结局的决定因素。
男性发病年龄小于女性(38 岁比 45 岁,p=0.05)。肌无力的起始部位为远侧上肢(61%)或远侧下肢(34%),偶尔在上臂(5%)。三分之一的患者初始诊断为运动神经元病。8%的患者存在腱反射活跃但无病理反射。在最常受累的尺神经(80%)和正中神经(77%)中发现传导阻滞,但偶尔仅在 Erb 点和腋窝之间(6%)或肌皮神经(1%)中发现。94%的患者对 IVIg 治疗有反应:无反应者在首次治疗前的疾病持续时间较长(p=0.03)。76%的患者在本研究时正在接受 IVIg 维持治疗(中位治疗时间 6 年;范围 0-17 年):多年来,每周的剂量从 12 克增加到 17 克(p<0.01)。更严重的肌无力和残疾的独立决定因素是轴索丢失(p<0.001;p<0.0001)和未经 IVIg 治疗的疾病持续时间更长(p=0.03;p=0.07)。
本研究的结果可能有助于识别 MMN 的临床特征。早期 IVIg 治疗可能有助于延缓轴突变性和永久性损伤。
本研究提供了 IV 级证据,表明 IVIg 可改善 94%(95%置信区间,86.8%-97.4%)MMN 患者的肌肉力量和残疾(定义为至少 2 个肌群的肌力增加≥1 级,而其他肌群的肌力无下降)。