有枕部爆发活动的儿童期癫的临床特征

The clinical characteristics of childhood epilepsy with occipital paroxysm

目的 有枕部爆发活动的儿童期癫 (childhoodepilepsywithoccipitalparoxysms,CEOP)被分成两种综合征 ,即早发型和晚发型。本文总结了 1组 5 8例有枕部爆发活动的儿童癫的临床特征来探讨这一分类的临床意义。方法 5 8例 1996～ 2 0 0 0年在癫中心就诊的患儿 ,并需符合以下标准 :① 13岁以前发病 :②发病前的发育正常 ;③神经影像学检查无异常发现 ;④脑电图有枕部爆发活动 ,背景活动正常。结果 根据发作期的表现分为 3组。第 1组有发作期视觉症状 ,共 18例 (31% ) ,第 2组有强直性眼球偏转 ,共 2 7例 (4 7% ) ;第 3组既无视觉症状也无眼球偏转 ,为全身强直阵挛发作 ,共 13例 (2 2 % )。第 1组所有 18例病人均符合Gastaut型的诊断 ,发作短暂而频繁 ,6 1%的病人在白天发作。第 2组所有 2 7例符合Palayiotopoulos型的诊断 ,均有发作期眼球偏转 ,其中 33%的病人出现发作期呕吐 ,发作次数较少 ,15 %的病人仅有 1次发作 ,6 7%的病人仅在夜间发作 ,其发病年龄要早于Gastaut型 (P<0 0 1)。结论 Panayiotopoulos型是最常见的有枕部爆发活动的儿童癫。这两种综合征的诊断并不困难 ,因为它们具有完全不同的临床表现。

Objective In a recent proposal, the Commission on Classification and Terminology of the International League Against Epilepsy classified childhood epilepsy with occipital paroxysms(CEOP) into two syndromes with different seizure types: early onset (Panayiotopoulos type) and late onset (Gastaut type). We documented the clinical features of a cohort of 58 cases with CEOP to discuss the clinical significance of the classification. Methods All the patients visited the Epilepsy Center of PUMCH between 1996～2000 and fulfilled the following criteria: age onset of epilepsy before 13 years old; normal development before the onset of epilepsy; no neuroimaging abnormalities and EEG findings of normal background activity with occipital paroxysms. The medical records of those were reviewed. Results Three group were defined according to the predominant ictal manifestation. Group 1 consisted of 18 patients (31%) with ictal visual symptoms. Group 2 consisted of 27 patients(47%) with tonic eye deviation. Group 3 (nonvisual, nonadversive) consisted of 13 patients (22%) with generalized tonic-clonic seizures. Two syndromes were identified. The Gastaut type included all 18 patients in group 1; seizures were brief and frequent and were diurnal in 61%. The Panayiotopoulos type included all 27 patients in group 2; ictal eye deviation occurred in 100% of patients and ictal vomiting in 33%; Seizures were infrequent; 15% of patients had a single seizure and 67% had nocturnal seizures. Onset was earlier than for the Gastaut type(P<0 01). Conclusions The Panayiotopoulos-type is the most common type of CEOR. It is not difficult to diagnose the two syndromes due to their entirely different manifestations.