Iannetti P, Spalice A, Raucci U, Atzei G, Cipriani C
Department of Pediatrics (VII), University La Sapienza, Roma, Italy.
Brain Dev. 1997 Sep;19(6):418-21. doi: 10.1016/s0387-7604(97)00042-9.
Gelastic epilepsy, or ictal laughter, is a relatively uncommon type of seizure which may occur singly or, more frequently, with other types of convulsions. Gelastic seizures have been observed to be associated with many different conditions, mainly hypothalamic hamartomas. We report on a patient whose ictal laughter was the only neurologic disturbance. Ictal video-EEG demonstrated seizure arising from the left frontal region with subsequent involvement of the contralateral homologous area and secondary generalization. MRI showed an enlarged left frontal horn of the lateral ventricle. Postictal SPECT, performed 6 min after the seizure had ended, showed hypoperfusion in the bilateral frontoparietal region and in both cerebellar hemispheres; the presence of this abnormality may be due to the spreading of the cortical epileptogenic focus and to the complex intercommunication between the frontal cortex and the cerebellar hemispheres. Interictal SPECT, in accordance with MRI features, demonstrated a left frontoparietal hypoperfusion. The neurofunctional features observed in the reported child could suggest that gelastic epilepsy originates in the frontal cortex. However, further studies are undoubtedly needed to define the pathogenetic mechanisms of ictal laughter.
痴笑性癫痫，即发作性大笑，是一种相对罕见的癫痫发作类型，可单独出现，或更常见地与其他类型的惊厥一起出现。已观察到痴笑性癫痫发作与许多不同病症相关，主要是下丘脑错构瘤。我们报告了一名患者，其发作性大笑是唯一的神经功能障碍。发作期视频脑电图显示癫痫发作起源于左侧额叶区域，随后累及对侧同源区域并继发全面性发作。磁共振成像显示侧脑室左额角增大。癫痫发作结束6分钟后进行的发作后期单光子发射计算机断层扫描显示双侧额顶叶区域和双侧小脑半球灌注不足；这种异常情况的出现可能是由于皮质癫痫病灶的扩散以及额叶皮质与小脑半球之间复杂的相互联系。发作间期单光子发射计算机断层扫描根据磁共振成像特征显示左侧额顶叶灌注不足。在所报告儿童中观察到的神经功能特征可能提示痴笑性癫痫起源于额叶皮质。然而，无疑需要进一步研究来确定发作性大笑的发病机制。
