Correlation of clinical features and developmental assessment in patients of epilepsy secondary to Sturge-Weber syndrome

doi:10.19854/j.cnki.1008-2425.2025.01.0001

Abstract

Abstract: Objective To analyze the factors that may affect the cognitive status of patients with secondary-epilepsy with Sturge-Weber syndrome(SWS) and improve the understanding of this disease. Methods The clinical data of 10 patients who underwent preoperative evaluation with secondary epilepsy of SWS in the Epilepsy Center of Yuquan Hospital of Tsinghua University were retrospectively analyzed,including age at seizure onset,age at seizure onset,age at seizure onset,age at seizure onset,age at seizure onset,age at seizure onset,age at seizure onset,age at seizure onset.ASO),duration of epilepsy,type of SWS,range of leptomeningeal capillary malformation(LCM),type of epilepsy,history of epileptic state and cluster state,etc.According to the number of epileptoid discharges in the interseizure period,EEG scores were performed(0~3 points).The number of lobes involved in LCM was determined by Magnetic resonance imaging(MRI).Epileptic seizure status score(0~3) within 1 year prior to preoperative evaluation;All patients underwent cognitive assessment(0~3 points).Griffiths scale was used for those <8 years old at the time of preoperative assessment,and Wechsler Intelligence Scale for Children was used for those >8 years old. Results Ten patients were continuously included,and the median age of onset of epilepsy was 7.5 months.The correlation coefficient between age of onset of epilepsy and cognitive assessment was 0.36(P=0.31).The correlation coefficient between epilepsy duration and cognitive evaluation was -0.46(P=0.18).The correlation coefficient between the number of affected lobes and cognitive assessment was 0.66,P=0.04,indicating that the range of LCM was positively correlated with cognitive impairment.The correlation coefficient between seizure score and cognitive evaluation was 0.67(P=0.04),which was statistically significant,suggesting a positive correlation between seizure frequency and cognitive impairment. Conclusion Epilepsy in patients with SWS starts at an early age and is at a critical stage of nervous system development,which may adversely affect cognitive outcomes.The larger the LCM range and the higher the seizure frequency of SWS patients,the worse the cognitive outcomes of patients.Therefore,early control of seizures in SWS patients and reducing the frequency of seizures may play an important role in improving the cognitive level of patients.

Key words: Sturge-Weber syndrome, Clinical features, Seizure characteristics, Eeg score, Seizure score, Cognitive assessment, Correlation

摘要： 目的分析Sturge-Weber 综合征(Sturge-Weber syndrome,SWS)继发癫痫患者可能影响认知状况的因素,提高对此类疾病的认识。方法回顾性分析在清华大学玉泉医院癫痫中心术前评估过的10例SWS继发癫痫患者的临床资料,包括癫痫起病年龄(age at seizure onset ,ASO)、癫痫病程、SWS分型及软脑膜毛细血管畸形(leptomeningeal capillary malformation,LCM)范围、癫痫发作类型、是否有癫痫持续状态和丛集状态史等。根据发作间期癫痫样放电数量进行脑电图评分(0分~3分);根据磁共振成像 (magnetic resonance imaging,MRI)确定LCM累及脑叶数量;根据术前评估之前1年内癫痫发作情况评分(0分~3分);所有患者进行认知评估(0分~3分),术前评估时年龄<8岁者采用Griffiths量表,年龄>8岁者采用Wechsler儿童智力量表。结果连续性纳入10例患者,癫痫起病年龄中位数7.5月龄。癫痫起病年龄与认知评估相关系数为0.36,P=0.31。癫痫病程与认知评估相关系数为-0.46,P=0.18。受累脑叶数量与认知评估相关系数为0.66,P=0.04,说明LCM范围与认知受损呈正相关;癫痫发作评分与认知评估相关系数为0.67,P=0.04,有统计学意义,提示癫痫发作频率与认知受损呈正相关。结论 SWS患者的癫痫起病年龄较早,处于神经系统发育的关键阶段,对认知结果可能产生不利影响。SWS患者LCM范围越大、癫痫发作频率越高,患者的认知结果越差。因此,尽早控制SWS患者的癫痫发作、降低发作频率,可能对改善患者的认知水平起重要作用。

关键词: Sturge-Weber综合征, 临床特征, 癫痫发作特点, 脑电评分, 癫痫发作评分, 认知评估, 相关性

CLC Number:

R742.1

Ding Ying, Zhang Shen, Xu Guowei, Zhang Xuelian, Li Haijing, Zhou wenjing, Shi Jie. Correlation of clinical features and developmental assessment in patients of epilepsy secondary to Sturge-Weber syndrome[J]. Chinese Journal of Stereotactic and Functional Neurosurgery, 2025, 38(1): 1-5.

丁莹, 张申, 徐国卫, 张雪莲, 李海静, 周文静, 史洁. Sturge-Weber综合征继发癫痫的临床特征和认知结果相关性分析[J]. 立体定向和功能性神经外科杂志, 2025, 38(1): 1-5.

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