Acquired motor speech disorders in childhood epilepsy.

Michael Eyre,Steve Rose,Rachel Gwynn,Ronit M Pressler,Maria Clark
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Abstract

AIM To evaluate a group of children with epilepsy and motor speech regression, with the aim of characterizing their speech disorders, electrographic features, and outcomes. METHOD Children referred to a tertiary developmental epilepsy clinic with epilepsy and motor speech regression were identified retrospectively. A clinical history was taken, and longitudinal speech and cognitive data were recorded. Speech samples were scored for severity and speech features. Seizure frequency and epileptiform discharges in the interictal electroencephalogram were analysed. RESULTS Eighteen children (10 female) were evaluated, including seven with Landau-Kleffner syndrome and six with Rasmussen syndrome. Speech regression occurred at a mean age of 5 years (SD = 2 years 6 months), which was concurrent with seizure onset or peak seizure burden in eight children. Speech features included dysarthria (n = 13), phonological errors (n = 7), and dyspraxia (n = 6). Electrographic abnormalities occurred most frequently in the left centrotemporal and right frontal regions. Among children who were followed up, intelligibility of speech was affected in 13 at baseline and seven at follow-up (p = 0.03). Expressive language standardized scores increased from a mean (SD) of 50.0 (11.3) to 91.4 (27.8) in children with Landau-Kleffner syndrome (mean change = 41.4, 95% confidence interval [CI] 0.04-82.8, p = 0.0498) and decreased from 75.2 (15.3) to 59.0 (9.8) in children with Rasmussen syndrome (mean change -16.2, 95% CI -9.0 to -23.4, p = 0.002) over the follow-up. INTERPRETATION Motor speech disorders in epilepsy were severe, multifarious, and often fluctuated with seizure burden. Symptoms typically improved, especially in children with Landau-Kleffner syndrome, but rarely fully resolved.
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儿童癫痫的后天运动言语障碍。
目的对一组患有癫痫和运动性言语退步的儿童进行评估,以了解他们的言语障碍、电图特征和治疗结果。方法对转诊到三级发育性癫痫诊所的患有癫痫和运动性言语退步的儿童进行回顾性鉴别。采集临床病史,记录纵向言语和认知数据。对言语样本的严重程度和言语特征进行评分。对发作频率和发作间期脑电图中的痫样放电进行了分析。结果共评估了 18 名儿童(10 名女性),其中 7 名患有兰道-克莱夫纳综合征,6 名患有拉斯穆森综合征。言语障碍发生在平均 5 岁(SD = 2 岁 6 个月)时,其中 8 名儿童的言语障碍与癫痫发作开始或发作高峰期同时出现。言语特征包括构音障碍(13 例)、语音错误(7 例)和发音障碍(6 例)。电图异常最常发生在左侧颞中部和右侧额叶区域。在接受随访的儿童中,13 名儿童的语言清晰度在基线时受到影响,7 名儿童在随访时受到影响(P = 0.03)。兰道-克莱夫纳综合症患儿的语言表达能力标准化评分从平均值(标清)50.0(11.3)增至91.4(27.8)(平均变化=41.4,95%置信区间[CI] 0.04-82.8,P=0.0498),而语言表达能力标准化评分则从75.2(15.在随访期间,Rasmussen 综合征患儿的运动言语障碍从 75.2(15.3)降至 59.0(9.8)(平均变化为 -16.2,95% 置信区间 [CI] -9.0 至 -23.4,p = 0.002)。症状通常会有所改善,尤其是在患有兰道-克莱夫纳综合征的儿童中,但很少能完全缓解。
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