Iannetti Paola, Spalice Alberto, Rocchi Valerio, Verrotti Alberto
Department of Pediatrics, Division of Child Neurology, La Sapienza University of Rome, Viale Regina Elena 324, Rome, Italy.
J Child Neurol. 2009 Apr;24(4):472-6. doi: 10.1177/0883073808325656. Epub 2008 Dec 10.
Panayiotopoulos syndrome is a common and benign childhood autonomic epilepsy of debated localization. Although officially considered as occipital epilepsy, this is most likely of multifocal origin. Ictal electroencephalography is the gold standard of seizure localization, but in Panayiotopoulos syndrome, because patients have single or rare seizures, only 7 cases with ictal electroencephalography have been reported. Ictal onsets show variable anterior and more often posterior locations. We describe an 8-year-old girl with 5 nocturnal autonomic seizures typical of Panayiotopoulos syndrome from age 4. The last seizure was captured with electroencephalography and showed a diffuse onset of the ictal discharge, whereas various interictal electroencephalography had infrequent multifocal spikes. This case contributes to the understanding of the pathophysiology of Panayiotopoulos syndrome in favor of a diffuse and multifocal cortical epileptogenicity that triggers an unstable central autonomic nervous system solely or prior to the focal cortical symptoms.
潘纳约托普洛斯综合征是一种常见的儿童良性自主性癫痫,其起源部位存在争议。尽管官方将其视为枕叶癫痫,但它很可能起源于多灶性。发作期脑电图是癫痫发作定位的金标准,但在潘纳约托普洛斯综合征中,由于患者发作次数单一或稀少,仅有7例发作期脑电图的报道。发作起始部位显示出前部位置多变,而后部位置更为常见。我们描述了一名8岁女孩,从4岁起就出现了5次典型的潘纳约托普洛斯综合征夜间自主性发作。最后一次发作通过脑电图记录,显示发作期放电呈弥漫性起始,而不同的发作间期脑电图有罕见的多灶性棘波。该病例有助于理解潘纳约托普洛斯综合征的病理生理学,支持弥漫性和多灶性皮质致痫性,这种致痫性单独或在局灶性皮质症状出现之前触发不稳定的中枢自主神经系统。