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Assessment of motor unit loss in patients with spinal muscular atrophy
Clinical Neurophysiology ( IF 4.7 ) Pub Date : 2020-06-01 , DOI: 10.1016/j.clinph.2020.01.018
Boudewijn T H M Sleutjes 1 , Camiel A Wijngaarde 1 , Renske I Wadman 1 , Louise A M Otto 1 , Fay-Lynn Asselman 1 , Inge Cuppen 2 , Leonard H van den Berg 1 , W Ludo van der Pol 1 , H Stephan Goedee 1
Affiliation  

OBJECTIVE To assess motor unit (MU) changes in patients with spinal muscular atrophy (SMA) using compound muscle action potential (CMAP) scans. METHODS We performed CMAP scan recordings in median nerves of 24 treatment-naïve patients (median age 39; range 12-75 years) with SMA types 2-4. From each scan, we determined maximum CMAP amplitude (CMAPmax), a motor unit number estimate (MUNE), and D50 which quantifies the largest discontinuities within CMAP scans. RESULTS Median CMAPmax was 8.1 mV (range 0.9-14.6 mV), MUNE was 29 (range 6-131), and D50 was 25 (range 2-57). We found a reduced D50 (<25) in patients with normal CMAPmax (n = 12), indicating MU loss and enlarged MUs due to reinnervation. Lower D50 values were associated with decreased MUNE (P < 0.001, r = 0.68, n = 43). CMAPmax, MUNE and D50 values differed between SMA types (P < 0.001). Lower motor function scores were related to patients with lower CMAPmax, MUNE and D50 values (P < 0.001). CONCLUSIONS The CMAP scan is an easily applicable technique that is superior to routine assessment of CMAPmax in SMA. SIGNIFICANCE The detection of pathological MU changes across the spectrum of SMA may provide important biomarkers for evaluating disease course and monitoring treatment efficacy.

中文翻译:

脊髓性肌萎缩患者运动单位损失的评估

目的 使用复合肌肉动作电位 (CMAP) 扫描评估脊髓性肌萎缩症 (SMA) 患者的运动单位 (MU) 变化。方法 我们对 24 名 2-4 型 SMA 患者(中位年龄 39;范围 12-75 岁)的正中神经进行了 CMAP 扫描记录。从每次扫描中,我们确定了最大 CMAP 振幅 (CMAPmax)、运动单位数量估计值 (MUNE) 和 D50,它量化了 CMAP 扫描中的最大不连续性。结果 CMAPmax 中位数为 8.1 mV(范围 0.9-14.6 mV),MUNE 为 29(范围 6-131),D50 为 25(范围 2-57)。我们发现 CMAPmax 正常的患者(n = 12)的 D50 降低(<25),表明由于再神经支配导致 MU 丢失和 MU 增大。较低的 D50 值与降低的 MUNE 相关(P < 0.001,r = 0.68,n = 43)。CMAPmax、MUNE 和 D50 值在 SMA 类型之间存在差异(P < 0。001)。较低的运动功能评分与较低 CMAPmax、MUNE 和 D50 值的患者相关(P < 0.001)。结论 CMAP 扫描是一种易于应用的技术,优于 SMA 中 CMAPmax 的常规评估。意义 在 SMA 谱中检测病理性 MU 变化可能为评估病程和监测治疗效果提供重要的生物标志物。
更新日期:2020-06-01
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