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Stereo-Electro-Encephalo-Graphy SEEG With Robotic Assistance in the Presurgical Evaluation of Medical Refractory Epilepsy: A Technical Note
Published on: June 13, 2016
Seizure, Motor, and Cognitive Outcomes After Epilepsy Surgery for Patients With Sturge-Weber Syndrome: Results From a
Shu Wang1, Qing-Zhu Liu1, Rui Zhao1
1From the Department of Neurosurgery (S.W., X.W., J. Zhou, G.L., Y.G.), SanBo Brain Hospital, Capital Medical University; Department of Neurosurgery (S.W., K.Z., J. Zhang), Beijing Tiantan Hospital, Capital Medical University; Pediatric Epilepsy Center (Q.-Z.L., L.C.), Peking University First Hospital, Beijing; Department of Neurosurgery (R.Z.), Children's Hospital of Fudan University, Shanghai; Department of Neurosurgery (X.Z.), Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders; Epilepsy Center (S. Li), Guangdong Sanjiu Brain Hospital, Guangzhou; Department of Neurosurgery (Z.Y.), Xiangya Hospital, Central South University, Changsha, Hunan; Department of Neurosurgery (Y.S.), Xuanwu Hospital, Capital Medical University; Department of Neurosurgery (K.M., Y.L.), Capital Institute of Pediatrics, Beijing; Department of Neurosurgery (J.H.), Huashan Hospital, Fudan University, Shanghai; Department of Epilepsy Center (L.S.), The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangdong; Department of Neurosurgery (H.C.), The First Affiliated Hospital of Anhui Medical University, Hefei; Department of Neurology (X.L.), Affiliated ZhongDa Hospital, Southeast University, Nanjing, Jiangsu; Department of Neurology (J.S.), Affiliated Children's Hospital of Xi'an Jiaotong University, Shaanxi; Department of Neurosurgery (M.Z.), Henan Sanbo Brain Hospital, Zhengzhou; Department of Neurology (M.W., T.L.), SanBo Brain Hospital; and Department of Functional Neurosurgery (J. Zhang), Beijing Neurosurgical Institute, Capital Medical University; Beijing Key Laboratory of Neurostimulation (J. Zhang); Functional Neurosurgery Department (S. Liang), Beijing Children's Hospital, Capital Medical University, National Center for Children's Health; Beijing Key Laboratory of Epilepsy (G.L., Y.G.); and Center of Epilepsy (G.L., Y.G.), Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, China.
Background And Objectives:
Surgery is widely performed for refractory epilepsy in patients with Sturge-Weber syndrome (SWS), but reports on its effectiveness are limited. This study aimed to analyze seizure, motor, and cognitive outcomes of surgery in these patients and to identify factors associated with the outcomes.
Methods:
This was a multicenter retrospective observational study using data from patients with SWS and refractory epilepsy who underwent epilepsy surgery between 2000 and 2020 at 16 centers throughout China. Longitudinal postoperative seizures were classified by Engel class, and Engel class I was regarded as seizure-free outcome. Functional (motor and cognitive) outcomes were evaluated using the SWS neurologic score, and improved or unchanged scores between baseline and follow-up were considered to have stable outcomes. Outcomes were analyzed using Kaplan-Meier analyses. Multivariate Cox regression was used to identify factors associated with outcomes.
Results:
A total of 214 patients with a median age of 2.0 (interquartile range 1.2-4.6) years underwent surgery (focal resection, FR [n = 87]; hemisphere surgery, HS [n = 127]) and completed a median of 3.5 (1.7-5.0) years of follow-up. The overall estimated probability for being seizure-free postoperatively at 1, 2, and 5 years was 86.9% (95% CI 82.5-91.6), 81.4% (95% CI 76.1-87.1), and 70.7% (95% CI 63.3-79.0), respectively. The overall estimated probability of being motor stable at the same time post operatively was 65.4% (95% CI 58.4-71.2), 80.2% (95% CI 73.8-85.0), and 85.7% (95% CI 79.5-90.1), respectively. The overall probability for being cognition stable at 1, 2, and 5 years was 80.8% (95% CI 74.8-85.5), 85.1% (95% CI 79.3-89.2), and 89.5% (95% CI 83.8-93.2), respectively. Both FR and HS were effective at ensuring seizure control. For different HS techniques, modified hemispherotomy had comparable outcomes but improved safety compared with anatomical hemispherectomy. Regarding FR, partial resection (adjusted hazard ratio [aHR] 11.50, 95% CI 4.44-29.76), acute postoperative seizure (APOS, within 30 days of surgery; aHR 10.33, 95% CI 3.94-27.12), and generalized seizure (aHR 3.09, 95% CI 1.37-6.94) were associated with seizure persistence. For HS, seizure persistence was associated with APOS (aHR 27.61, 9.92-76.89), generalized seizure (aHR 7.95, 2.74-23.05), seizure frequency ≥30 times/month (aHR 4.76, 1.27-17.87), and surgical age ≥2 years (aHR 3.78, 1.51-9.47); motor stability was associated with severe motor defects (aHR 5.23, 2.27-12.05) and postoperative seizure-free status (aHR 3.09, 1.49-6.45); and cognition stability was associated with postoperative seizure-free status (aHR 2.84, 1.39-5.78) and surgical age <2 years (aHR 1.76, 1.13-2.75).
Discussion:
FR is a valid option for refractory epilepsy in patients with SWS and has similar outcomes to those of HS, with less morbidity associated with refractory epilepsy. Early surgical treatment (under the age of 2 years) leads to better outcomes after HS, but there is insufficient evidence that surgical age affects FR outcomes. These findings warrant future prospective multicenter cohorts with international cooperation and prolonged follow-up in better exploring more precise outcomes and developing prognostic predictive models.
Classification Of Evidence:
This study provides Class IV evidence that in children with SWS and refractory seizures, surgical resection-focal, hemispherectomy, or modified hemispherotomy-leads to improved outcomes.
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