Objective

To develop and test wearable monitoring of surface electromyography and motion for detection and quantification of positive and negative myoclonus in patients with progressive myoclonic epilepsy type 1 (EPM1).

Methods

Surface electromyography and three-dimensional acceleration were measured from 23 EPM1 patients from the biceps brachii (BB) of the dominant and the extensor digitorum communis (EDC) of the non-dominant arm for 48 hours. The patients self-reported the degree of myoclonus in a diary once an hour. Severity of myoclonus with action was evaluated by using video-recorded Unified Myoclonus Rating Scale (UMRS). Correlations of monitored parameters were quantified with the UMRS scores and the self-reported degrees of myoclonus.

Results

The monitoring-based myoclonus index correlated significantly (p < 0.001) with the UMRS scores (ρ = 0.883 for BB and ρ = 0.823 for EDC) and with the self-reported myoclonus degrees (ρ = 0.483 for BB and ρ = 0.443 for EDC). Ten patients were assessed as probably having negative myoclonus in UMRS, while our algorithm detected that in twelve patients.

Conclusions

Wearable monitoring was able to detect both positive and negative myoclonus in EPM1 patients.

Significance

Our method is suitable for quantifying objective, real-life treatment effects at home and progression of myoclonus.

中文翻译：

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1 型进行性肌阵挛癫痫中阳性和阴性肌阵挛的可穿戴监测

客观的

开发和测试表面肌电图和运动的可穿戴监测，用于检测和量化 1 型进行性肌阵挛癫痫 (EPM1) 患者的阳性和阴性肌阵挛。

方法

表面肌电图和三维加速度从 23 名 EPM1 患者的优势臂肱二头肌 (BB) 和非优势臂的伸肌 (EDC) 测量 48 小时。患者每小时在日记中自我报告肌阵挛的程度。使用视频记录的统一肌阵挛评定量表 (UMRS) 评估动作肌阵挛的严重程度。监测参数的相关性通过 UMRS 评分和自我报告的肌阵挛程度进行量化。

结果

基于监测的肌阵挛指数 与 UMRS 评分（ BB 的ρ = 0.883 和 EDC 的ρ = 0.823）以及自我报告的肌阵挛程度（ BB 的ρ = 0.483 和 EDC 的ρ = 0.443 ）显着相关（p < 0.001））。10 名患者在 UMRS 中被评估为可能具有阴性肌阵挛，而我们的算法在 12 名患者中检测到了这一点。

结论

可穿戴监测能够检测到 EPM1 患者的阳性和阴性肌阵挛。

意义

我们的方法适用于量化客观、真实的家庭治疗效果和肌阵挛的进展。