Beaumanoir A, Mira L, van Lierde A
Fondazione Mariani, Milan, Italy.
Brain Dev. 1996 Mar-Apr;18(2):139-41. doi: 10.1016/0387-7604(95)00112-3.
Epileptic seizures induced by sudden movement and paroxysmal kinesigenic choreoathetosis (PKC) have often been confused in the past, owing to the close similarity of the attacks, the equally good response to anticonvulsants, and the frequent occurrence of epilepsy and PKC in the same family, or even in the same patient. The pathophysiology of PKC is still unclear and its relationship with epilepsy open to discussion. The sparing of consciousness and the lack of postictal phenomena are constant features of PKC, thus differentiating this syndrome from epilepsy. We report the case of an 8-year-old boy with frequent brief tonic attacks, without loss of consciousness, triggered by sudden movement. The neurologic examination, EEG and MRI did not help to differentiate between epilepsy and PKC. Only the occurrence of a longer seizure with clouding of consciousness and the recording of the postictal abnormalities on the EEG supported a diagnosis of reflex epilepsy induced by movement.
过去,由突然运动诱发的癫痫发作和阵发性运动诱发性舞蹈手足徐动症(PKC)常常被混淆,这是因为发作表现极为相似、对抗惊厥药物的反应同样良好,以及癫痫和PKC在同一家族甚至同一患者中频繁出现。PKC的病理生理学仍不清楚,其与癫痫的关系也有待探讨。意识保留和发作后现象的缺失是PKC的恒定特征,借此可将该综合征与癫痫区分开来。我们报告了一例8岁男孩的病例,他频繁出现由突然运动触发的短暂强直性发作,且无意识丧失。神经系统检查、脑电图(EEG)和磁共振成像(MRI)均无助于区分癫痫和PKC。只有出现一次伴有意识模糊的较长发作以及EEG上记录到发作后异常,才支持运动诱发的反射性癫痫的诊断。