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目的探讨不典型儿童良性癫癎伴中央颞区棘波(BECT)患儿的临床特点、认知、脑电图特点及患儿对治疗的反应。方法分析及随访9例(男5例,女4例;起病年龄3岁~8岁2个月,平均5岁3个月。起病前精神运动发育正常)诊断为不典型BECT患儿的临床特点、智力、认知、脑电图特点及对治疗的反应。结果 9例中4例出现脑电图慢波睡眠电持续状态(ESES),并伴有发作频率增加及明显的神经精神症状,经抗癫癎药物(AEDs)治疗发作控制,3例脑电图得到改善,但3例患儿仍伴有较明显的行为及认知问题。1例出现癫癎性失语,还表现有淡漠、孤独症样表现伴脑电图恶化,经AEDs治疗发作及脑电图明显改善,随访3 a仍少语、表达困难。2例病程中出现难以控制的频繁局限运动性发作,伴注意力缺陷及多动,脑电图放电频率增多,对多种AEDs反应不佳,但1.0 a及1.5 a后自然缓解。2例出现不典型失神,伴有精神行为症状,亦伴脑电图恶化,换用丙戊酸或加用氯硝西泮后发作控制,脑电图好转,精神行为症状部分改善。结论 BECT存在较为不典型的复杂多样的演变过程,因存在认知障碍方面的缺陷,其良性预后应受到挑战。
Objective To investigate the clinical features, cognitive and electroencephalographic features of children with benign epilepsy with central temporal spikes (BECT) and the response to treatment in children. Method analysis and follow-up 9 cases (5 males and 4 females; onset age 3 years old to 8 years old 2 months, average 5 years old 3 months before onset of mental motor development normal) diagnosis of atypical BECT children Clinical characteristics, intelligence, cognition, EEG characteristics and response to treatment. Results In 9 of 9 patients, there was EEG slow wave sleep state (ESES) accompanied by increased seizure frequency and obvious neuropsychiatric symptoms, seizures controlled by antiepileptic drugs (AEDs), 3 cases of electroencephalogram However, three children still had more obvious behavioral and cognitive problems. 1 case of epileptic aphasia, but also showed indifference, autism-like performance with deterioration of the EEG, AEDs treatment of seizures and EEG was significantly improved, follow-up 3 a still less words, the expression is difficult. Two cases of uncontrolled frequent locomotor seizures were found in 2 patients. With attention deficit hyperactivity and multiple motions, the frequency of EEG discharges increased, which did not respond well to many AEDs. However, spontaneous remission was achieved at 1.0 and 1.5 years. 2 cases of atypical absence, with mental symptoms, but also with the deterioration of electroencephalogram, valproic acid or chlorpromazine for seizure control, electroencephalogram improved, part of the mental and behavioral symptoms improved. Conclusions There is a rather atypical complex and diverse evolutionary process of BECT. Due to the defects of cognitive impairment, its benign prognosis should be challenged.