Emory University, Atlanta, Georgia, USA.
Department of Rehabilitation and Therapy Services, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
Muscle Nerve. 2018 Nov;58(5):729-732. doi: 10.1002/mus.26319. Epub 2018 Oct 2.
Clinical and electrophysiological studies to measures disease activity in juvenile myasthenia gravis (JMG) are limited.
Retrospective review of the clinical profile, Myasthenia Gravis Foundation of America (MGFA) scores, serial stimulated jitter analysis (Stim-JA) of the orbicularis oculi muscle, grip strength, and spirometry of patients with JMG who were followed in a multidisciplinary clinic was performed.
Thirteen patients with JMG (9 females) with mean age of 13.2 ± 4.8 years and follow-up duration of 25.3 ± 8.3 months (range, 6-39) with ≥ 2 Stim-JA recordings were included. The mean jitter, mean percentage of apparent single-fiber action potentials (%ASFAP) with increased jitter, and mean %ASFAP with blocking at baseline values (77.3 ± 54.7 µs, 64.3% ± 35.8%, 39% ± 38.6%, respectively) and at follow-up (53 ± 45.4 µs, 51.2% ± 34.5%, 17% ± 29.4%, respectively) were abnormal; however, no statistically significant interval difference was noted. The electrophysiological data correlated significantly with Myasthenia Gravis Foundation of America (MGFA) class. Grip strength and spirometry did not correlate with MGFA class.
Stimulated jitter values are sensitive biomarkers in JMG. Muscle Nerve 58: 729-732, 2018.
针对青少年重症肌无力（JMG）疾病活动的临床和电生理研究有限。
对在多学科诊所中接受随访的 JMG 患者的临床特征、美国重症肌无力基金会（MGFA）评分、眼轮匝肌连续刺激颤搐分析（Stim-JA）、握力和肺活量进行回顾性分析。
共纳入 13 例 JMG 患者（9 名女性），平均年龄为 13.2±4.8 岁，随访时间为 25.3±8.3 个月（范围 6-39 个月），≥2 次 Stim-JA 记录。平均颤搐值、平均有增宽颤搐的单个纤维动作电位百分比（%ASFAP）和平均有阻滞的 %ASFAP 值在基线时（77.3±54.7µs、64.3%±35.8%、39%±38.6%）和随访时（53±45.4µs、51.2%±34.5%、17%±29.4%）均异常；然而，差异无统计学意义。电生理数据与 MGFA 分级显著相关。握力和肺活量与 MGFA 分级无相关性。
刺激颤搐值是 JMG 的敏感生物标志物。《神经肌肉疾病》58:729-732,2018.
