儿童良性癫痫伴中央颞区棘波的变异型

目的：探讨儿童良性癫痫伴中央颞区棘波（BECT）变异型的电－临床特征、治疗及神经心理学预后。方法：对病程中出现失神样发作、粗大震颤、运动不协调（Ⅰ型）或言语及口部运动障碍（Ⅱ型）等变异型症状的BECT儿童进行系列视频脑电图（VEEG）监测，并对其发作表现、电生理特征、神经心理学损伤及对抗癫痫药物治疗的反应进行分析和随访。结果：9例患儿脑电图（EEG）均显示清醒和睡眠期大量Rolandic区棘慢波发放，慢波睡眠期棘慢波指数在50 ％～85 ％。Ⅰ型变异型症状中，5例失神样发作时EEG为继发双侧同步性2～3 Hz棘慢波节律暴发， 6例粗大震颤患儿经直立伸臂试验证实有局部负性肌阵挛发作，Ⅱ型变异型症状中，6例患儿有言语表达障碍，其中5例有口咽部失用，3例完全失语。2例患儿兼有Ⅰ型和Ⅱ型的电－临床表现。7例患儿有各种神经心理学损伤，Ⅰ型症状者更严重。6例患儿经丙戊酸单用或与氯硝西泮联合治疗有效，3例加用肾上腺皮质激素治疗后症状改善。结论：BECT变异型均伴有明显的EEG恶化。Ⅰ型主要表现为继发性不典型失神和负性肌阵挛发作，神经心理学预后不良；Ⅱ型以口咽部失用为特征，预后较好。丙戊酸、苯二氮卓类药物及肾上腺皮质激素治疗对改善BECT变异型的电－临床症状有效。

The variants of benign childhood epilepsy with centrotemporal spikes

【Abstract】 Objective To research the electro-clinical characteristics, therapeutic response and prognosis of neuropsychology in the children with variants of benign childhood epilepsy with centrotemporal spikes (BECT). Methods Performing video-EEG monitoring for children with BECT who had some variant symptoms including absence-like seizures, jerks, incoordinate movement (Ⅰtype), or speech and oromotor deficits (Ⅱtype). Analyzing the seizures semeiology, electrophysiologic features, neuropsychologic impairments and responses for antiepileptic drugs (AEDs). Results The electroencephalography (EEG) of all 9 cases showed abundance of spike and waves (SW) in rolandic areas during wake and sleep. The SW index were as high as fifty per cent to eighty-five per cent. Ⅰtype variant symptoms included absence-like seizures with secondary bilateral synchronous 2-3Hz spike-wave rhythms during ictal EEG in 5 cases, negative myoclonic seizures confirmed by test of standing-up and extending arms forward in 6 patients. Ⅱ type variant symptoms represented as speech expression disorders in 6 children, five of them had oral-pharynx apraxia and three cases had aphasia. Two patients had both Ⅰtype and Ⅱ type symptoms. Neuropsychologic impairment occurred in seven cases, and appeared more severity in the patients withⅠtype than Ⅱtype variant. Valproic acid only or combined with clonazepam were effective in 6 cases. The clinical and EEG problems were improved in the other three by adding on steroid therapy. Conclusion The variants of BECT often associated with EEG deteriorated. Ⅰtype primarily represents as secondary atypical absences and negative myoclonus ,often with poor neuropsychologic prognosis. A notable characteristic in Ⅱ type is oral-pharynx apraxia and with related good prognosis. Valproic acid, benzodiazepines and adrenocorical hormone were effective for improvement of electro-clinical manifenstation of BECT variants.