Department of Neurology, Division of Neuromuscular Disease, Mount Sinai School of Medicine, New York, New York, USA.
Muscle Nerve. 2011 Apr;43(4):537-42. doi: 10.1002/mus.21914. Epub 2011 Feb 8.
Are there electrophysiological findings that predict response to intravenous immunoglobulin (IVIg) in patients with lower motor neuron (LMN) syndromes without multifocal conduction block (MCB)?
We enrolled 9 patients with LMN syndromes without MCB to receive 18 weeks of IVIg therapy. Response was measured at weeks 2 and 18 using the Appel Amyotrophic Lateral Sclerosis (AALS) score (includes grip and pincer strength measures), ALS Functional Rating Scale (ALSFRS), and electrophysiological measures, including motor unit estimates (MUNEs).
No change occurred in AALS or ALSFRS scores posttreatment. Grip/pincer strength increased in 7 patients (P = 0.028) after initial treatment (responders); 2 showed no improvement (non-responders). No electrophysiological measure changed after treatment in either group but MUNEs trended higher (P = 0.055). "Abnormal A-waves" (complex, repetitive biphasic, or present in multiple nerves) occurred in pretreatment studies more often in responders (P = 0.028).
"Abnormal A-waves" may signal IVIg-responsive LMN syndromes even if conduction block is absent.
是否存在电生理学发现可以预测无多灶性传导阻滞（MCB）的下运动神经元（LMN）综合征患者对静脉注射免疫球蛋白（IVIg）的反应？
我们招募了 9 名患有 LMN 综合征且无 MCB 的患者，接受 18 周的 IVIg 治疗。使用 Appel 肌萎缩侧索硬化症（AALS）评分（包括握力和指尖力量测量）、肌萎缩侧索硬化症功能评定量表（ALSFRS）和电生理学测量，包括运动单位估计（MUNEs），在第 2 周和第 18 周评估反应。
治疗后 AALS 或 ALSFRS 评分无变化。7 名患者（P = 0.028）在初始治疗后（应答者）握力/指尖力量增加；2 名患者无改善（无应答者）。两组的任何电生理测量值在治疗后均无变化，但 MUNEs 呈上升趋势（P = 0.055）。在治疗前研究中，应答者更常出现“异常 A 波”（复杂、重复的双相或存在于多条神经中）（P = 0.028）。
即使不存在传导阻滞，“异常 A 波”也可能预示着对 IVIg 有反应的 LMN 综合征。
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