Ros Leandra A A, Sleutjes Boudewijn T H M, Stikvoort García Diederik J L, Goedee H Stephan, Asselman Fay-Lynn, van den Berg Leonard H, van der Pol W Ludo, Wadman Renske I
Department of Neurology, University Medical Center Utrecht, UMC Utrecht Brain Center, Utrecht University, Utrecht, The Netherlands.
Clin Neurophysiol Pract. 2023 Jul 4;8:123-131. doi: 10.1016/j.cnp.2023.06.001. eCollection 2023.
Electrophysiological techniques are emerging as an aid in identifying prognostic or therapeutic biomarkers in patients with spinal muscular atrophy (SMA), but electrophysiological assessments may be burdensome for patients. We, therefore, assessed feasibility and tolerability of multimodal peripheral non-invasive electrophysiological techniques in a cohort of patients with SMA.
We conducted a single center, longitudinal cohort study investigating the feasibility and tolerability of applying multimodal electrophysiological techniques to the median nerve unilaterally. Techniques consisted of the compound muscle action potential scan, motor nerve excitability tests, repetitive nerve stimulation and sensory nerve action potential. We assessed tolerability using the numeric rating scale (NRS), ranging from 0 (no pain) to 10 (worst possible pain), and defined the protocol to be tolerable if the NRS score ≤ 3. The protocol was considered feasible if it could be performed according to test and quality standards.
We included 71 patients with SMA types 1-4 (median 39 years; range 13-67) and 63 patients at follow-up. The protocol was feasible in 98% of patients and was well-tolerated in up to 90% of patients. Median NRS score was 2 (range 0-6 at baseline and range 0-4 at follow-up (p < 0.01)). None of the patients declined follow-up assessment.
Multimodal, peripheral, non-invasive, electrophysiological techniques applied to the median nerve are feasible and well-tolerated in adolescents and adults with SMA types 1-4.
Our study supports the use of non-invasive multimodal electrophysiological assessments in adolescents and adults with SMA types 1-4.
电生理技术正逐渐成为识别脊髓性肌萎缩症（SMA）患者预后或治疗生物标志物的辅助手段，但电生理评估对患者来说可能负担较重。因此，我们评估了多模式外周非侵入性电生理技术在一组SMA患者中的可行性和耐受性。
我们进行了一项单中心纵向队列研究，调查单侧正中神经应用多模式电生理技术的可行性和耐受性。技术包括复合肌肉动作电位扫描、运动神经兴奋性测试、重复神经刺激和感觉神经动作电位。我们使用数字评分量表（NRS）评估耐受性，范围从0（无疼痛）到10（最严重的疼痛），并将NRS评分≤3定义为该方案可耐受。如果该方案能够按照测试和质量标准执行，则认为是可行的。
我们纳入了71例1-4型SMA患者（中位年龄39岁；范围13-67岁）和63例接受随访的患者。该方案在98%的患者中可行，在高达90%的患者中耐受性良好。NRS评分中位数为2（基线时范围为0-6，随访时范围为0-4（p<0.01））。没有患者拒绝随访评估。
应用于正中神经的多模式、外周、非侵入性电生理技术在1-4型SMA的青少年和成人中是可行的且耐受性良好。
我们的研究支持在1-4型SMA的青少年和成人中使用非侵入性多模式电生理评估。
