Musaeus Christian Sandøe, Pedersen Jette Stokholm, Kjær Troels Wesenberg, Johannsen Peter, Waldemar Gunhild, Haverberg Maria Joy Normann, Bacher Theis, Nielsen Jørgen Erik, Roos Peter
Danish Dementia Research Centre (DDRC), Rigshospitalet and Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark.
Department of Neurology, Zealand University Hospital, Roskilde, Denmark.
Front Aging Neurosci. 2021 Sep 13;13:714220. doi: 10.3389/fnagi.2021.714220. eCollection 2021.
A rare cause of inherited frontotemporal dementia (FTD) is a mutation in the gene on chromosome 3 leading to the autosomal dominantly inherited FTD (-FTD). Since -FTD is clinically well-characterized, and patients show a distinct pattern of executive dysfunction, the condition offers possible insight in the early electroencephalographic (EEG) changes in the cortical networks. Specifically, EEG microstate analysis parses the EEG signals into topographies believed to represent discrete network activations. We investigated the EEG dynamics in patients with symptomatic -FTD ( = 5) as well as pre-symptomatic mutation carriers ( = 5) compared to non-carrier family members ( = 6). The data was parsed into four archetypal microstates and global power was calculated. A trend was found for lower occurrence in microstate D in -FTD (value = 0.177, value = 2.036). Patients with recent symptom onset (<1 year) showed an increased duration of microstate D, whereas patients who had been symptomatic for longer periods (>2 years) showed decreased duration. Patients with -FTD present with executive dysfunction, and microstate D has previously been shown to be associated with the fronto-parietal network. The biphasic pattern may represent the pathophysiological changes in brain dynamics during neurodegeneration, which may apply to other neurodegenerative diseases.
遗传性额颞叶痴呆(FTD)的一种罕见病因是3号染色体上的基因突变,导致常染色体显性遗传的FTD(-FTD)。由于-FTD在临床上有明确特征,且患者表现出独特的执行功能障碍模式,该病症为了解皮质网络早期脑电图(EEG)变化提供了可能。具体而言,EEG微状态分析将EEG信号解析为被认为代表离散网络激活的地形图。我们研究了有症状的-FTD患者(=5)以及症状前突变携带者(=5)与非携带者家庭成员(=6)相比的EEG动态变化。数据被解析为四个原型微状态,并计算了全局功率。发现-FTD中微状态D的出现频率较低(值=0.177,值=2.036)。近期出现症状(<1年)的患者微状态D的持续时间增加,而有症状较长时间(>2年)的患者微状态D的持续时间减少。-FTD患者存在执行功能障碍,先前已证明微状态D与额顶叶网络有关。这种双相模式可能代表神经退行性变期间脑动力学的病理生理变化,这可能适用于其他神经退行性疾病。