Junming Zhu, Yuanyuan Zhao, Fang Feng, Weiming Fu, Ryan Hays, Jianmin Zhang, Li Feng, Xiao Jin, Shuda Chen
The Second Affiliated Hospital, Zhejiang University College of Medicine, Department of Neurosurgery, Hangzhou, China.
Turk Neurosurg. 2014;24(1):70-4. doi: 10.5137/1019-5149.JTN.6362-12.1.
Congenital bilateral perisylvian syndrome (CBPS) is characterized by epilepsy, cognitive deficits, pseudobulbar palsy and diplegia of the facial, pharyngeal and masticatory muscles. Epilepsy has been described in nearly 90% of affected patients. The epilepsy is usually severe and pharmacoresistant in about 55 percent of CBPS patients. Until now, only 12 cases of surgical treatment on CBPS have been reported; the surgical treatment is usually corpus callosotomy. In this paper, we describe a previously unreported combination of anterior corpus callosotomy plus anterior temporal lobectomy with amygdalohippocampectomy for a patient with CBPS, resulting in a satisfactory clinical outcome. Based on this case, we suggest that palliative focal resective surgery combined with anterior corpus callosotomy should be considered when a predominance of the epileptiform discharges suggests focal onset in patients with CBPS. Meanwhile, the clinical decision to adopt this combination surgery must be based on a thorough pre-surgical evaluation, and should take into account the clinical, radiological, and EEG features.
先天性双侧外侧裂周综合征（CBPS）的特征为癫痫、认知缺陷、假性延髓麻痹以及面部、咽部和咀嚼肌的双侧瘫。近90%的受累患者有癫痫发作。在约55%的CBPS患者中，癫痫通常较为严重且药物难治。截至目前，仅报道了12例CBPS的手术治疗病例；手术治疗通常为胼胝体切开术。在本文中，我们描述了1例CBPS患者采用先前未报道的胼胝体前部切开术加颞叶前部切除术及杏仁核海马切除术的联合手术，取得了满意的临床效果。基于该病例，我们建议，当CBPS患者癫痫样放电以局灶性发作为主时，应考虑姑息性局灶性切除手术联合胼胝体前部切开术。同时，采用这种联合手术的临床决策必须基于全面的术前评估，并应考虑临床、影像学和脑电图特征。
