Wearable monitoring of positive and negative myoclonus in progressive myoclonic epilepsy type 1

Abstract

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.

Keywords: Accelerometry; Myoclonus; Progressive myoclonic epilepsy type 1 (EPM1); Surface electromyography (EMG); Unverricht-Lundborg disease; Wearable monitoring.