Caraballo Roberto Horacio, Cersósimo Ricardo Oscar, Fejerman Natalio
Hospital de Pediatría Prof. Dr. Juan P Garrahan. Buenos Aires, Argentina.
Epileptic Disord. 2005 Dec;7(4):341-6.
We report on two girls and one boy with clinical and electroencephalographic features of late-onset childhood epilepsy with occipital paroxysms of the "Gastaut type", showing an unusual evolution. Neurological examination and brain imaging were normal in all three. At the age of 7.5 years, eight years and ten years respectively, the three children presented with episodes of visual symptoms when awake, and in one of them, the seizures were occasionally followed by oculocephalic deviation. The interictal EEG showed bilateral occipital spike-wave activated by eye closing. In two patients, the occipital seizures had been immediately followed by typical absences, since onset; in the other patient, five months after onset. The ictal EEG showed irregular bilateral occipital spike-wave discharges during the visual symptoms, followed by generalized spike-wave activity during the typical absences. The typical absences were activated by hyperventilation; the EEG did not show continuous spikes and waves during slow sleep. These three patients, with typical electroclinical features of "Gastaut type", childhood occipital epilepsy, demonstrated an evolution which, to our knowledge, has not been previously described. We investigated whether this unusual, age-dependent evolution was due to secondary bilateral synchrony or if these electroclinical features represent two types of idiopathic epileptic syndromes in the same patients.
我们报告了两名女孩和一名男孩，他们具有“加斯东型”晚发性儿童枕叶癫痫的临床和脑电图特征，呈现出不寻常的演变过程。三名患者的神经学检查和脑部影像学检查均正常。这三名儿童分别在7.5岁、8岁和10岁时，清醒时出现视觉症状发作，其中一名患者的发作偶尔会伴有眼球-头偏向。发作间期脑电图显示闭眼可激活双侧枕叶棘波。两名患者自发病起，枕叶发作后立即出现典型失神发作；另一名患者在发病五个月后出现。发作期脑电图显示视觉症状期间双侧枕叶不规则棘波放电，随后在典型失神发作期间出现全身性棘波活动。典型失神发作可由过度换气诱发；脑电图在慢波睡眠期未显示持续性棘慢波。这三名具有“加斯东型”儿童枕叶癫痫典型电临床特征的患者，呈现出一种据我们所知此前未被描述过的演变过程。我们研究了这种不寻常的、与年龄相关的演变是否是由于继发性双侧同步化，或者这些电临床特征是否代表同一患者中的两种特发性癫痫综合征类型。
