早发型良性儿童枕叶癫痫18例脑电图及预后分析

A study of clinical features,EEG and prognosis of patients with early-onset benign childhood occipital epilepsy

目的探讨早发型良性儿童枕叶癫痫(EOBCOE)的临床发作形式、痫性放电波形与分布、病理生理特征、临床诊断方法及预后演变规律。方法对2005年1月至2008年12月重庆医科大学附属儿童医院神经内科诊疗的18例EOBCOE患儿资料进行回顾性临床分析,对其清醒和睡眠脑电图(EEG)资料及治疗随访结果进行研究。结果18例患儿首次起病年龄2岁2个月至8岁6个月。5例分别有癫痫家族史和(或)热性惊厥家族史,4例有热性惊厥史。EOBCOE具有突出的自主神经性发作及迟后的特殊运动发作症状(面色苍白和发作性呕吐、头眼斜视旋转发作或凝视、继发性部分或全面性发作、自主神经性癫痫持续状态)。2例伴Rolandic发作。多数1至数月发作1次,个别频繁发作。EEG示13例有枕区或枕区为主局灶或多灶性棘-慢波放电,波形以高幅宽大棘-慢波为主,也可见不典型小尖波,均可被睁眼所抑制。发作期显示枕区或额区为主或全脑节律性棘-慢波活动。患儿对卡马西平、丙戊酸钠和妥泰等疗效较好;发作基本消失11例,明显减轻4例,病情波动2例,猝死1例。EEG痫性放电减轻8例,变化不明显4例,正常5例。除7例有轻度学习困难和行为异常以外,其他患儿神经精神发育基本正常。结论EOBCOE是一种早期起病的良性儿童枕叶或自主神经性癫痫,具有突出的自主神经性症状,局灶或继发全面性发作或癫痫持续状态等临床特征与其他良性儿童癫痫相关,EEG示枕区或枕外多灶性放电,多数预后良好。

Objective To investigate the clinical seizure patterns, morphology and topography of epileptic discharges and prognosis of Early-Onset Benign Childhood Occipital Epilepsy （EOBCOE）. Methods According to the criteria of EOBCOE made by ILAE （2001 ）, eighteen patients with EOBCOE were retrospectively followed up and the data analyzed regarding clinical manifestation, interietal and ictal EEG during awakeness and sleep as well as therapeutic efficacy of 4 years. Results The onset of seizures occurred at the age between 2 years and ：2 months and 8 years and 6 months with a mean age of 4.7 yrs. Five of eighteen cases had a family history of epilepsy and/or febrile seizures, one of whom was iden- tiffed as the idiopathic epilepsy ; 4 cases had febrile seizures. The remarkable autonomic seizures and delayed specific motor seizures were the clinical hallmark of EOBCOE, including pallor and ictal vomiting, followed by the eye and head deviation, and unilateral or generalized motor seizures, and even autonomic status epilepticus associated with a partial impairment of consciousness. Two cases had Rolandic seizures including speech arrest, hemifacial spasms and salivation. Most of cases had sporadic seizures with free-intervals of 1-6 months or longer, but some of them still had frequent seizures. EEG showed 13 cases had occipital or occipital predominance of mnhifocal epileptic discharges including focal oc- cipital spikes in 9 cases, the occipito-temporo-parietal or occipito-frontal spikes in 4 cases;4 had extraoccipital spikes, including Rolandic spikes or frontal predominance of paroxysmal generalized discharges in 2 cases respectively, lnterictal spikes were typically of high voltage of polyphasic spike-waves and easily blocked by eye opening, lctal EEG showed the occipital or frontal predominance and generalized rhythmic spike-wave complex. Patients with EOBCOE were responsive In CBZ, VPA and TPM. Seizures ceased in 11 cases, were partially controlled and fluctuated in 4 and 2 cases respective-ly; one died of sudden unexplained death. The abnormal EEGs were improved in 8, unchanged in 4 and became normal in 5 cases. All patients developed normally except 7 who were found to have mild learning and behavourproblems. Conclusion EOBCOE is characterized by paroxysmal autonomic symptoms and signs, focal or generalized motor seizures or autonomic status epilepticns, which is associated with other benign childhood focal seizures and epilepsy. EEGs show the occipital predominance o1＂ extraoccipital spikes of mnhifoci. Prognosis is generally good.