Repetitive wide-band cortical power in benign adult familial myoclonus epilepsy

IF 3.4 3区医学 Q1 CLINICAL NEUROLOGY

Clinical Neurophysiology Pub Date : 2026-02-01 Epub Date: 2025-12-19 DOI:10.1016/j.clinph.2025.2111486

Haruo Yamanaka , Katsuya Kobayashi , Takefumi Hitomi , Maya Tojima , Masao Matsuhashi , Kiyohide Usami , Ryosuke Takahashi , Akio Ikeda

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引用次数: 0

Abstract

Background

Cortical tremor (CT), a rhythmic variant of cortical myoclonus (CM), is the hallmark of benign adult familial myoclonus epilepsy (BAFME), though the underlying mechanism of rhythmicity remains unproven. This study aimed to reveal the cortical rhythmic activity of CT using induced activity analysis for somatosensory evoked potentials (SEP).

Methods

We investigated 46 SEP datasets from 23 patients (11 with BAFME, 12 with other CM) and 35 SEPs from 18 healthy controls. SEPs were recorded by 1.1-Hz stimuli at a sampling rate of 10,000 Hz. A short-time Fourier transform was applied to each SEP epoch, and the power spectrums were averaged. We set an analysis window of 0–150 ms and a frequency range of 0–1,000 Hz for time–frequency representation and compared the induced power changes between groups.

Results

Stimulus-induced power changes over a wide-band (0–1000 Hz) were conspicuously prominent in BAFME patients compared to both CM and controls. These activities presented repetitive and alternating increases and decreases in power and its total number of induced activities were the highest in age 40s and declined with aging.

Conclusions

We demonstrated rhythmic cortical activity in BAFME patients, which may reflect the underlying pathophysiology of CT.

Significance

Detecting the induced activity of a single somatosensory stimulus may offer novel insights into the pathophysiology of BAFME.

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良性成人家族性肌阵挛性癫痫的重复宽带皮质功率

背景皮质震颤（CT）是皮质肌阵挛（CM）的一种节律变体，是良性成人家族性肌阵挛性癫痫（BAFME）的标志，尽管节律性的潜在机制尚未得到证实。本研究旨在利用体感诱发电位（SEP）的诱发活动分析来揭示CT皮层的节律性活动。方法对23例BAFME患者（11例BAFME， 12例其他CM）的46个SEP数据集和18例健康对照的35个SEP数据集进行分析。在1.1 Hz的刺激下，以10,000 Hz的采样率记录sep。对每个SEP历元进行短时傅里叶变换，对功率谱进行平均。我们设置了0-150 ms的分析窗口和0-1,000 Hz的频率范围来表示时间-频率，并比较了组间诱导功率的变化。结果与CM和对照组相比，BAFME患者在宽频带（0-1000 Hz）上刺激引起的功率变化明显突出。这些活动的强度呈反复、交替的增减趋势，诱导活动总数在40岁时最高，随年龄增长而下降。结论BAFME患者有节律性皮层活动，这可能反映了CT的潜在病理生理。意义检测单个体感刺激的诱导活性可能为BAFME的病理生理学提供新的见解。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical Neurophysiology 医学-临床神经学

CiteScore

8.70

自引率

6.40%

发文量

932

审稿时长

59 days

期刊介绍： As of January 1999, The journal Electroencephalography and Clinical Neurophysiology, and its two sections Electromyography and Motor Control and Evoked Potentials have amalgamated to become this journal - Clinical Neurophysiology. Clinical Neurophysiology is the official journal of the International Federation of Clinical Neurophysiology, the Brazilian Society of Clinical Neurophysiology, the Czech Society of Clinical Neurophysiology, the Italian Clinical Neurophysiology Society and the International Society of Intraoperative Neurophysiology.The journal is dedicated to fostering research and disseminating information on all aspects of both normal and abnormal functioning of the nervous system. The key aim of the publication is to disseminate scholarly reports on the pathophysiology underlying diseases of the central and peripheral nervous system of human patients. Clinical trials that use neurophysiological measures to document change are encouraged, as are manuscripts reporting data on integrated neuroimaging of central nervous function including, but not limited to, functional MRI, MEG, EEG, PET and other neuroimaging modalities.