Khomand Payam, Katzberg Hans, Ngo Mylan, Bril Vera
Ellen and Martin Prosserman Centre for Neuromuscular Diseases, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada.
Case Rep Neurol. 2020 Jan 22;12(1):40-44. doi: 10.1159/000505234. eCollection 2020 Jan-Apr.
Electrophysiological studies are essential for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP), but the utility of nerve conduction studies in monitoring outcomes in individual CIDP patients is controversial. Electrophysiological improvements after short-term treatment have been described in large cohorts of CIDP patients, but the magnitude of the changes is small and might be obscured in individual patients due to the variation inherent in nerve conduction testing. We present the case of a CIDP patient treated successfully with immunosuppression and followed for 31 years with serial standardized clinical and electrophysiological evaluations. Improvement in electrophysiological parameters lagged clinical changes for up to 2 years, but all motor parameters improved (distal motor and F wave latencies, conduction velocities, and compound muscle action potential amplitudes) even with evidence of initial axonal damage. Worsening of electrophysiological parameters, specifically increasing F wave latencies, heralded clinical relapse by as much as a year. Electrophysiological parameters do improve with treatment in CIDP patients, although the changes can take up to 2 years, and also worsening electrophysiological parameters can herald clinical relapse and might help guide therapeutic decisions.
电生理研究对于慢性炎症性脱髓鞘性多发性神经病(CIDP)的诊断至关重要,但神经传导研究在监测个体CIDP患者预后方面的实用性存在争议。在大量CIDP患者队列中已描述了短期治疗后的电生理改善情况,但变化幅度较小,且由于神经传导测试固有的变异性,个体患者的变化可能会被掩盖。我们报告一例CIDP患者,经免疫抑制治疗成功,并通过系列标准化临床和电生理评估随访31年。电生理参数的改善比临床变化滞后长达2年,但即使有初始轴索损伤的证据,所有运动参数(远端运动和F波潜伏期、传导速度和复合肌肉动作电位波幅)均有所改善。电生理参数的恶化,特别是F波潜伏期延长,可预示临床复发长达一年。CIDP患者的电生理参数确实会随着治疗而改善,尽管变化可能需要长达2年时间,而且电生理参数恶化也可预示临床复发,并可能有助于指导治疗决策。