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¹The Second Affiliated Hospital, Zhejiang University College of Medicine, Department of Neurosurgery, Hangzhou, China
²Zhejiang Provincial People’s Hospital, Department of Neurosurgery, Hangzhou, China
³UT Southwestern Medical Center, Department of Neurology and Neurotherapeutics, Dallas, USA 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. Keywords : Congenital bilateral perisylvian syndrome (CBPS), Anterior corpus callosotomy, Anterior temporal resection, Epilepsy, Amygdalohippocampectomy