Pesántez-Ríos Gabriela, Martínez-Bermejo Antonio, Arcas Joaquín, Merino-Andreu Milagros, Ugalde-Canitrot Arturo
UAM. Universidad Autonoma de Madrid. Hospital Universitario La Paz, 28046 Madrid, Espana.
Rev Neurol. 2015 Aug 1;61(3):106-13.
The development of atypical features in rolandic epilepsy is part of a clinical spectrum of phenotypes that are variable, idiopathic and age-dependent, as well as having a genetically determined predisposition.
To study the electroclinical characteristics suggesting an atypical development in rolandic epilepsy.
A retrospective search was performed in 133 children diagnosed with atypical benign focal epilepsy (ABFE), Landau-Kleffner syndrome and continuous spike-wave during sleep (CSWS). Nine patients were selected, all of whom presented atypical clinical features and an electroencephalogram (EEG) pattern of electrical status epilepticus during sleep (ESES) in the course of their rolandic epilepsy.
The average age at onset of rolandic epilepsy was 5 years. Patients showed a deterioration of both their clinical features and their EEG recording one and a half years later, on average. ABFE was observed in three of them and CSWS in six. No cases of Landau-Kleffner syndrome were found. The EEG in wakefulness showed the focus to be in the left centrotemporal region in six patients and in three of them it was on the right-hand side. All the patients presented ESES in the EEG during sleep. An atypical pattern was observed in the regional ESES in three of the patients. Moreover, cognitive and behavioural disorders were detected due to deficits in specific learning areas, such as language, memory, attention and restlessness.
The early onset of rolandic epilepsy, the appearance of new seizures with an increased frequency and the frontocentrotemporal focus in the EEG, which increases in frequency, both in wakefulness and in sleep, are all electroclinical characteristics of an atypical development.
罗兰多癫痫非典型特征的发展是临床表型谱的一部分,这些表型具有变异性、特发性、年龄依赖性,并且有遗传决定的易感性。
研究提示罗兰多癫痫非典型发展的电临床特征。
对133例诊断为非典型良性局灶性癫痫(ABFE)、Landau-Kleffner综合征和睡眠期持续性棘慢波(CSWS)的儿童进行回顾性研究。选取9例患者,他们在罗兰多癫痫病程中均表现出非典型临床特征及睡眠期癫痫性电持续状态(ESES)的脑电图(EEG)模式。
罗兰多癫痫的平均发病年龄为5岁。患者平均在发病一年半后临床特征及EEG记录均出现恶化。其中3例为ABFE,6例为CSWS。未发现Landau-Kleffner综合征病例。清醒时EEG显示病灶位于左侧中央颞区的有6例患者,位于右侧的有3例。所有患者睡眠期EEG均出现ESES。3例患者的局部ESES出现非典型模式。此外,由于语言、记忆、注意力和多动等特定学习领域的缺陷,检测到认知和行为障碍。
罗兰多癫痫的早发、新发癫痫发作频率增加以及EEG中额中央颞区病灶在清醒和睡眠时频率均增加,这些都是非典型发展的电临床特征。