双侧裂综合征一例

Congenital bilateral perisylvian syndrome(CBPS) is rare in literature,especially in China.In this article,we report the clinical and treatment of a patient with CBPS and discuss its mechanism,clinical features and therapy.This patient was a 28-year-old man.His main clinical features were pseudobulbar palsy,cognitive deficits and intractable epilepsy.MRI shows bilateral thickening of the cortex around the sylvian fissures which were deeper than normal and polymicrogyria.The electroencephalogram demonstrated slow spike in right temporal lobe and left frontal lobe.Rhythmal 4 Hz θ waves exist in left frontal and parietal lobe.As the epilepsy was poorly controlled by antiepileptic,section of the corpus callosum was carried out.After callosotomy,there was pronounced seizure reduction and intelligence development improvement.CBPS is characterized by pseudobulbar palsy,cognitive deficits,and bilateral perisylvian abnormalities in imaging studies.If intractable epilepsy is combined,callosotomy may be effective.

先天性双侧外侧裂综合征(附4例报告)

先天性双侧外侧裂综合征是一种少见的以假性球麻痹、癫痫、智力低下和双侧外侧裂区皮质发育异常为特征的神经发育障碍。本文4例根据临床表现和影像学检查诊断为该综合征的患者,其中男3人,女1人,年龄范围12～26岁。4例患者均因癫痫发作就诊,影像学检查均显示双侧外侧裂皮质发育异常。临床表现为轻至中度的智力障碍、言语发育迟缓、构音障碍,咽反射消失和轻度锥体束征等。对于临床上表现为癫痫、假性球麻痹、智力低下、影像学提示双侧外侧裂萎缩的患者应想到先天性双侧外侧裂综合征的可能性。