Caraballo Roberto, Cersósimo Ricardo, Fejerman Natalio
Hospital Nacional de Pediatria Juan P. Garrahan, Buenos Aires, Argentina.
Epilepsia. 2007 Jun;48(6):1054-61. doi: 10.1111/j.1528-1167.2007.01085.x. Epub 2007 Apr 18.
To characterize the electroclinical features and evolution of Panayiotopoulos Syndrome (PS).
Children with electroclinical criteria of PS were prospectively identified and followed-up clinically, and with sleep and awake EEGs between February 1990 and 2006.
We identified 192 patients with PS. In the same length of time 398 children with benign childhood epilepsy with centro-temporal spikes (BCECTS) were registered. PS had a peak age at onset of 5 years. Autonomic manifestations were one of the most common ictal event. Ictal deviation of the eyes and progression to generalized convulsions were also quite frequent. Approximately one third had partial status epilepticus. In all patients except five, the seizures occurred during sleep. One-third also had fits while awake. Sixteen children had concomitant symptoms of rolandic epilepsy and eight developed rolandic seizures after remission of PS seizures. Prognosis was excellent. Eighty-four (44.2%) had a single seizure, 79 (41.2%) had 2-5 fits, and 28 (14.6%) had frequent seizures.
PS is less common than BCECTS, but is well defined and easily recognizable by clinical and EEG features, with autonomic manifestations as one of the most common ictal event.
描述潘纳约托普洛斯综合征（PS）的临床电生理特征及演变过程。
前瞻性地确定符合PS临床电生理标准的儿童，并于1990年2月至2006年期间对其进行临床随访，同时进行睡眠和清醒脑电图检查。
我们共确定了192例PS患者。在同一时期，登记了398例伴有中央颞区棘波的儿童良性癫痫（BCECTS）患者。PS的发病高峰年龄为5岁。自主神经表现是最常见的发作事件之一。发作时眼球偏斜并进展为全身性惊厥也较为常见。约三分之一的患者出现部分性癫痫持续状态。除5例患者外，所有患者的发作均发生在睡眠期间。三分之一的患者在清醒时也会发作。16名儿童伴有罗兰多癫痫的症状，8名儿童在PS发作缓解后出现罗兰多癫痫发作。预后良好。84例（44.2%）患者仅有一次发作，79例（41.2%）患者发作2 - 5次，28例（14.6%）患者发作频繁。
PS比BCECTS少见，但通过临床和脑电图特征易于明确诊断和识别，自主神经表现是最常见的发作事件之一。
