van der Veen Sterre, Maliepaard Amber, van der Stouwe Madelein, Dalenberg Jelle, Tuitert Inge, Elting Jan Willem J, Tijssen Marina A J
Department of Neurology, University of Groningen, University Medical Centre Groningen (UMCG), Groningen, The Netherlands.
Expertise Centre Movement Disorders Groningen, University Medical Centre Groningen (UMCG), Groningen, The Netherlands.
Mov Disord. 2024 Dec;39(12):2275-2280. doi: 10.1002/mds.29990. Epub 2024 Sep 24.
Myoclonus is characterized by involuntary, shock-like movements, of which cortical (CM) and non-cortical myoclonus (NCM) are most common. Electrophysiology can help differentiate between these subtypes; however, the diagnostic value of several features is largely unknown.
This study aims to determine the diagnostic value of the burst duration in distinguishing CM and NCM.
We manually identified the burst duration of 8 patients with CM, confirmed by electromyography-electroencephalography registration or somatosensory-evoked potentials, and 19 patients with NCM, suspected due to a myoclonus-dystonia phenotype (MYC/DYT-SGCE positive and negative).
The sensitivity and specificity were calculated to assess the diagnostic value. The burst duration of CM (31.1 ms) was significantly shorter than that of NCM (56.7 ms), with a sensitivity of 100% and a specificity of 89.5% at a threshold of 45.0 ms. A minimum of 10 randomly selected bursts were sufficient for reliable diagnostic accuracy.
The burst duration seems a valuable supportive diagnostic criterion for distinguishing CM and NCM.
肌阵挛的特征为不自主的、类似电击样的运动,其中皮质性肌阵挛(CM)和非皮质性肌阵挛(NCM)最为常见。电生理学有助于区分这些亚型;然而,几种特征的诊断价值在很大程度上尚不清楚。
本研究旨在确定爆发持续时间在区分CM和NCM中的诊断价值。
我们手动确定了8例经肌电图-脑电图记录或体感诱发电位证实的CM患者以及19例因肌阵挛-肌张力障碍表型(MYC/DYT-SGCE阳性和阴性)而疑似NCM的患者的爆发持续时间。
计算敏感性和特异性以评估诊断价值。CM的爆发持续时间(31.1毫秒)显著短于NCM(56.7毫秒),在阈值为45.0毫秒时,敏感性为100%,特异性为89.5%。至少随机选择10次爆发足以获得可靠的诊断准确性。
爆发持续时间似乎是区分CM和NCM的一项有价值的辅助诊断标准。