早发性和晚发性儿童良性枕叶癫痫的临床特征和脑电图改变

Analysis of clinical and electroencephalographic features in patients with early-and late-onset benign childhood epilepsy with occipital paroxysms

目的对比分析早发性儿童良性枕叶癫痫(EBOS)和晚发性儿童良性枕叶癫痫(LOS)的临床特点、EEG改变、治疗及预后,帮助临床诊治。方法30例患者,经临床和EEG诊断为枕部放电的儿童良性癫痫(BCEOP),再按2001年国际抗癫痫联盟建议,区分为EBOS组12例和LOS组18例,对比分析两组发病年龄、既往史、家族史、发作频率、持续时间及时间分布、临床特征、EEG改变及对药物治疗的反应。结果EBOS组平均发病年龄(5.6±2.4)岁,发作频率少而持续时间长,有夜间发作倾向;LOS组平均发病年龄(12.1±2.7)岁,发作频率高而持续时间短,视觉症状常见,可出现视幻觉,易继发全身强直-阵挛发作,白天发作为主。两组患者头眼偏转发作、跌倒发作及发作后头痛、呕吐症状无显著性差异(P>0.05)。两组发作间期EEG大部分示枕叶为主的棘、尖慢波,但EBOS组较局限且波形典型;LOS组易于泛化。EBOS对多种抗癫痫药物(AED)反应好,单药治疗均可控制发作;LOS对AED治疗反应差,5例(27.8%)无效。结论EBOS和LOS在发病年龄、发作频率及持续时间、发作时间分布、视觉症状、EEG放电特点、对AED治疗的反应等方面均有差别,综合分析上述特征可从临床上作鉴别诊断。

Objective To compare the clinical and electroencep halographic features and therapeutic responses between early onset benignoccipital seizure susceptibility syndrome （EBOS） and late onset childhood idiopathic occipital seizure （LOS）. Methods Thirty patients were diagnosed as benign childhood epilepsy with occipital paroxysms defined by the Commission of the International League Against Epilepsy （ILAE） in 1989. Twelve patients of them were characterized as having EBOS and 18 patients having LOS as described by ILAE proposal in 2001. The differences between EBOS and LOS with regard to age of onset, clinical data of seizures, EEG changes and therapeutic responses were studied. Results The mean age of onset of EBOS patients [（5.6±2.4） years] was significantly younger than that in LOS [ （12.1±2.7） years]. Compared with LOS patients, EBOS patients had less frequent seizures, longer seizure duration, and more nocturnal seizures. LOS patients had more visual symptoms （including hallucinations） and tended to secondary generalized tonic-clonic seizures that occurred mostly in daytime. The interictal EEG showed the spike- and sharp-show waves mainly in the occipital, and the waves were more typical and limited in EBOS patients than those in LOS ones. The discharges in the occipital of LOS patients were easy to generalization in EEG. EBOS was controlled well by AED monotherapy, but the monotherapy was ineffective in 5 LOS patients （27.8%）. Conclusion To distinguish EBOS from LOS, detailed description and analysis on the age of onset, the frequency and duration of seizures, visual symptoms, EEG changes and therapeutic responses are essential.