以癫痫发作为首要症状的可逆性胼胝体压部病变综合征患儿临床特征及预后影响因素分析

Analysis of Clinical Features and Prognostic Factors in Children with Reversible Splenial Lesion Syndrome with Epilepsy as the Primary Symptom

目的 探讨以癫痫发作为首要症状的可逆性胼胝体压部病变综合征(RESLES)患儿临床特征及预后影响因素。方法 选取2016年1月—2021年12月收治的以癫痫发作为首要症状的RESLES患儿143例,统计分析其临床特征和预后情况,根据预后情况将其分为预后不良组(35例)和预后良好组(108例),探讨其预后影响因素,构建Nomogram预测模型,并采用受试者工作特征(ROC)曲线和决策曲线(DCA)验证该模型对以癫痫发作为首要症状RESLES患儿预后不良的预测价值和临床效用。结果 143例中表现为惊厥发作者占86.71%(124/143);有前驱感染症状者占89.51%(128/143);头颅MRI检查T1WI低或稍低信号者占91.61%(131/143),DWI高或稍高、FLAIR高或稍高、T2WI高或稍高者均达100.00%(143/143),孤立胼胝体压部病变者占81.82%(117/143),病变形状斑片形占51.05%(73/143)。合并胼胝体其他部位病变、意识障碍、心肌型肌酸激酶同工酶(CK-MB)、胶质纤维酸性蛋白(GFAP)、β-肌动蛋白(β-actin)、细胞间黏附分子-1(ICAM-1)是以癫痫发作为首要症状RESLES患儿预后不良的危险因素(P<0.01)。ROC曲线分析显示Nomogram预测模型预测以癫痫发作为首要症状的RESLES患儿预后不良的曲线下面积为0.909,95%CI 0.849,0.951,DAC显示该模型具有良好的临床效用。结论 以癫痫发作为首要症状的RESLES患儿以惊厥发作及前驱感染症状为主要表现,头颅MRI检查T1WI呈低或稍低信号,DWI、FLAIR、T2WI呈高或稍高信号,合并胼胝体其他部位病变、意识障碍、CK-MB、GFAP、β-actin、ICAM-1是其预后不良影响因素,构建Nomogram预测模型有助于筛选其预后不良高危人群。

Objective To investigate the clinical features and prognostic factors of reversible splenial lesion syndrome(RESLES)with epilepsy as the primary symptom.Methods A total of 143 RESLES children with epilepsy as the primary symptom were selected from January 2016 to December 2021,and their clinical features and prognosis were statistically analyzed.According to the prognosis,they were divided into poor prognosis group(n=35)and good prognosis group(n=108).The factors influencing the prognosis of patients with RESLES were explored.A Nomogram prediction model was established,and the predictive value and clinical efficacy of this model were verified by receiver operating characteristic(ROC)curve and decision curve analysis(DCA).Results Among 143 cases,86.71%(124/143)had convulsion;89.51%(128/143)had symptoms of prodrome infection.In head MRI,91.61%(131/143)of patients had low or slightly low T1WI signal,100.00%(143/143)had high or slightly high DWI,high or slightly high FLAIR,high or slightly high T2WI,81.82%(117/143)had isolated splenial lesions,and 51.05%(73/143)had lesion shape plaques.The combination of extrascallosal lesions,disturbance of consciousness,myocardial creatine kinase isoenzyme(CK-MB),glial fibrillary acid protein(GFAP),β-actin,and intercellular adhesion molecule-1(ICAM-1)were risk factors for poor prognosis in children with RESLES with epilepsy as the primary symptom(P<0.01).ROC curve analysis showed that the area under the curve of Nomogram model for predicting poor prognosis of RESLES children with epilepsy as the primary symptom was 0.909,95%CI was 0.849,0.951,and DAC showed that this model had good clinical efficacy.Conclusion The main manifestations of RESLES with epilepsy as the primary symptom are convulsive attack and prodromal infection.Head MRI showed low or slightly low signal on T1WI,and high or slightly high signal on DWI,FLAIR and T2WI.Extrascallosum lesions,disturbance of consciousness,CK-MB,GFAP,β-actin,and ICAM-1 are the factors influencing the prognosis of the patients,and Nomogram prediction model is established to help screen the high-risk groups for poor prognosis.