Froelich J, Sassen R, Elger C E, Lehmkuhl G
Department of Child and Adolescent Psychiatry, University of Cologne, Germany.
Klin Padiatr. 2003 Mar-Apr;215(2):65-8. doi: 10.1055/s-2003-38502.
We report the case of a two years old boy showing gelastic fits as the leading clinical symptom of a rare complex cerebral malformation with closed-lip schizencephaly, an arachnoid cyst and a partial agenesis of the corpus callosum. After 5 uneventful interictal electroencephalograms the patient underwent 24 h EEG with telemetry while finally presenting two laughing fits with associated spike-wave discharges in the right-centroparietal region. Medical treatment with carbamacepine was initiated and the seizure frequency decreased significantly. This is the first cited case of gelastic fits occurring in schizencephaly. In contrast to previously cited cases with laughing fits we observed a good response to anticonvulsive treatment. Moreover our patient presented a surprisingly favourable psycho-mental and motor development during an 8 months observation period.
我们报告了一名两岁男孩的病例，其主要临床症状为痴笑发作，这是一种罕见的复杂脑畸形的表现，该畸形包括闭唇型脑裂畸形、蛛网膜囊肿和胼胝体部分发育不全。在5次发作间期脑电图正常后，患者接受了24小时遥测脑电图检查，最终出现两次痴笑发作，同时右侧中央顶叶区域伴有棘波放电。开始使用卡马西平进行药物治疗，癫痫发作频率显著降低。这是首次报道的脑裂畸形中出现痴笑发作的病例。与先前报道的伴有痴笑发作的病例不同，我们观察到抗惊厥治疗有良好效果。此外，在8个月的观察期内，我们的患者在心理和运动发育方面表现出惊人的良好状态。
