目的探讨脊髓动静脉瘘的治疗经验。方法回顾性分析1993～2010年我科收治的3例髓周动静脉瘘和13例硬脊膜动静脉瘘的临床资料。利用Aminoff and Logue评分评价手术前后的脊髓功能改变情况。结果16例均行脊髓血管造影检查和手术治疗，5例在脊髓血管造影检查后病情加重，11例无变化。术后运动功能：9例改善，7例无变化。排尿功能：13例出现排尿困难者中，11例改善，1例恶化，1例无变化。本组无手术并发症和临床复发。结论显微手术治疗硬脊膜动静脉瘘及I型髓周动静脉瘘刨伤小、简单易行、疗效确切。
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.
