儿童非酒精性韦尼克脑病4例临床分析

Non-alcoholic Wernicke encephalopathy in 4 children

目的分析4例非酒精性韦尼克脑病患儿的临床资料,探讨其临床特点及预后。方法4例非酒精性韦尼克脑病患儿,收集患儿临床资料,包括起病诱因、临床表现、既往史、实验室检查结果、头颅MRI检查结果、治疗情况等;随访2~6年,记录病情变化。结果4例患儿起病前均无明确诱因,1例既往有癫痫病史,3例无其他病史。4例患儿均有共济失调症状,并不同表现为嗜睡、精神差、哭声弱、双侧眼睑下垂、视物模糊、右侧眼球内斜视、右侧眼球震颤、呕吐、抽搐等。4例患儿头颅MRI均表现为典型的韦尼克脑病影像学改变,即丘脑、顶盖、中脑导水管周围不同程度的对称性长T_(2)信号。3例患儿起病至静脉补充维生素B_(1)时间分别为9、16、10d,剂量均为100mg/d,3d后临床症状均改善,8d后症状完全消失,随访4~6年未遗留后遗症;1例患儿起病2个月开始静脉补充维生素B_(1),100mg/d,共15d,临床症状无好转,规律康复治疗1年后仍有右侧眼球内斜视、眼球震颤、眼球运动不灵活和行走不稳等。结论儿童非酒精性韦尼克脑病临床表现多样、无特异性,可无明确诱因及基础疾病,头颅MRI影像学具有典型特征;早期诊断并及时静脉应用维生素B_(1)治疗可逆转神经系统症状。

Objective To analyze the clinical data of 4 children with non-alcoholic Wernicke encephalopathy(WE)and to investigate the clinical features and prognosis.Methods The clinical data of 4 children with non-alcoholic WE were collected,including the causes of disease onset,clinical manifestations,previous history,laboratory examination results,cranial MRI results and treatment,etc.The patients were followed up for 2 to 6 years to record the changes of disease.Results Non-alcoholic WE developed with unclear causes in 4 cases,1 case had a history of epilepsy,and the remaining 3 cases had no special history.Ataxia symptoms were found in all cases,and the other clinical manifestations included drowsiness,lethargy,decreased crying,bilateral eyelid ptosis,blurred vision,right eye esotropia,right nystagmus,vomiting or convulsions.All 4 cases had typical imaging changes of WE on MRI,as symmetry long T_(2) signal of different degrees around thalamus,tectum and aqueduct.Three cases received intravenous vitamin B_(1)supplementation by day 9,16 and 10 after onset,respectively,with the dose of 100 mg/d;their symptoms were improved after 3-d treatment,and disappeared completely after 8-d treatment;no sequel was found after 4 to 6 years of follow-up.One case started intravenous administration of vitamin B_(1)supplementation 2 months after onset,with the dose of 100 mg/d,but the clinical symptoms were not alliviated after 15-d treatment,and still had symtoms as right esotropia,nystagmus,immobility of eye movement and walking instability after 1-year regular rehabilitation treatment.Conclusions The clinical manifestations of non-alcoholic WE in children are diverse and nonspecific,with no clear causes or underlying diseases.Cranial MRI has typical imaging characteristics.Early diagnosis and timely intravenous administration of vitamin B_(1)can reverse the neurological symptoms.