Caraballo Roberto Horacio, Fortini Sebastian, Flesler Santiago, Pasteris María Constanza, Caramuta Luciana, Portuondo Ernesto
Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
Seizure. 2015 Feb;25:117-25. doi: 10.1016/j.seizure.2014.10.003. Epub 2014 Oct 13.
To retrospectively analyze the electroclinical characteristics, etiology, treatment, and prognosis of patients with epileptic encephalopathy with status epilepticus during sleep (ESES) with unusual EEG features and to corroborate if this series of patients is part of the ESES syndrome.
Charts of 17 patients with typical clinical manifestations of the ESES syndrome with focal ESES of non-REM sleep at onset and during the focal ESES phase, or bilateral synchronic and asynchronic ESES with a symmetric or asymmetric morphology, continuous or subcontinuous and sometimes multifocal paroxysms with or without slow-wave activity during slow sleep seen between 2000 and 2012 were analyzed.
Mean patient follow-up from onset was 7.5 years. An idiopathic cause was found in seven patients, a structural cause in eight, and etiology was unknown in the remaining two. The median age at onset of the unusual ESES syndrome was 7 years. During the ESES phase, 15 children developed new seizure types, negative myoclonus was observed in seven patients, positive myoclonus in five, and absences in nine. Six patients had motor impairment, two had auditory verbal agnosia, and two had motor speech impairment. Attention deficit hyperactivity disorder was observed in four, aggressiveness in six, memory deficit in two, and impaired temporospatial orientation in four. The patients with focal ESES in the frontal region showed behavioral disturbances and/or motor deterioration, and in those with temporo-occipital involvement the dominant clinical manifestations were language and/or behavioral disturbances.
Our patients with typical clinical manifestations of ESES syndrome but with unusual EEG patterns may be variants of this syndrome.
回顾性分析睡眠期癫痫性脑病伴癫痫持续状态（ESES）且脑电图特征异常患者的电临床特征、病因、治疗及预后，并证实这一系列患者是否属于ESES综合征。
分析2000年至2012年间17例具有ESES综合征典型临床表现的患者病历，这些患者在发作起始及局灶性ESES期非快速眼动睡眠期出现局灶性ESES，或双侧同步及非同步ESES，形态对称或不对称，慢睡眠期出现连续或亚连续、有时为多灶性发作，伴或不伴慢波活动。
患者自发病起的平均随访时间为7.5年。7例患者病因不明，8例为结构性病因，其余2例病因未知。异常ESES综合征的发病中位年龄为7岁。在ESES期，15例儿童出现新的发作类型，7例观察到负性肌阵挛，5例为正性肌阵挛，9例出现失神发作。6例患者有运动障碍，2例有听觉性言语失认，2例有运动性言语障碍。4例观察到注意力缺陷多动障碍，6例有攻击性，2例有记忆缺陷，4例有颞空间定向障碍。额叶局灶性ESES患者表现为行为障碍和/或运动功能恶化，颞枕叶受累患者的主要临床表现为语言和/或行为障碍。
我们的患者具有ESES综合征的典型临床表现，但脑电图模式异常，可能是该综合征的变异型。
