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Electrophysiological study of neuromuscular junction in congenital myasthenic syndromes, congenital myopathies, and chronic progressive external ophthalmoplegia
Neuromuscular Disorders ( IF 2.8 ) Pub Date : 2020-11-01 , DOI: 10.1016/j.nmd.2020.10.002
Vitor Marques Caldas 1 , Carlos Otto Heise 1 , João Aris Kouyoumdjian 2 , Antônio Alberto Zambon 1 , André Macedo Serafim Silva 1 , Eduardo de Paula Estephan 3 , Edmar Zanoteli 1
Affiliation  

This study was designed to analyze the sensitivity, specificity, and accuracy of jitter parameters combined with repetitive nerve stimulation (RNS) in congenital myasthenic syndrome (CMS), chronic progressive external ophthalmoplegia (CPEO), and congenital myopathies (CM). Jitter was obtained with a concentric needle electrode during voluntary activation of the Orbicularis Oculi muscle in CMS (n = 21), CPEO (n = 20), and CM (n = 18) patients and in controls (n = 14). RNS (3 Hz) was performed in six different muscles for all patients (Abductor Digiti Minimi, Tibialis Anterior, upper Trapezius, Deltoideus, Orbicularis Oculi, and Nasalis). RNS was abnormal in 90.5% of CMS patients and in only one CM patient. Jitter was abnormal in 95.2% of CMS, 20% of CPEO, and 11.1% of CM patients. No patient with CPEO or CM presented a mean jitter higher than 53.6 µs or more than 30% abnormal individual jitter (> 45 µs). No patient with CPEO or CM and mild abnormal jitter values presented an abnormal decrement. Jitter and RNS assessment are valuable tools for diagnosing neuromuscular transmission abnormalities in CMS patients. A mean jitter value above 53.6 µs or the presence of more than 30% abnormal individual jitter (> 45 µs) strongly suggests CMS compared with CPEO and CM.

中文翻译:

先天性肌无力综合征、先天性肌病和慢性进行性外眼肌麻痹的神经肌肉接头电生理研究

本研究旨在分析抖动参数联合重复神经刺激 (RNS) 在先天性肌无力综合征 (CMS)、慢性进行性外眼肌麻痹 (CPEO) 和先天性肌病 (CM) 中的敏感性、特异性和准确性。在 CMS (n = 21)、CPEO (n = 20) 和 CM (n = 18) 患者和对照组 (n = 14) 的眼轮匝肌的自愿激活过程中,使用同心针电极获得了抖动。对所有患者的六种不同肌肉(小指外展肌、胫前肌、斜方肌上部、三角肌、眼轮匝肌和鼻肌)进行 RNS(3 Hz)。90.5% 的 CMS 患者和仅 1 名 CM 患者的 RNS 异常。95.2% 的 CMS、20% 的 CPEO 和 11.1% 的 CM 患者出现抖动异常。没有 CPEO 或 CM 患者的平均抖动高于 53。6 µs 或超过 30% 的异常个体抖动 (> 45 µs)。没有 CPEO 或 CM 和轻度异常抖动值的患者出现异常减少。抖动和 RNS 评估是诊断 CMS 患者神经肌肉传递异常的宝贵工具。高于 53.6 µs 的平均抖动值或超过 30% 的异常个体抖动 (> 45 µs) 强烈表明 CMS 与 CPEO 和 CM 相比。
更新日期:2020-11-01
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