儿童小脑性缄默综合征临床特点  

Clinical characteristics of cerebellar mutism syndrome in children

作　　者：杨伟 葛明[1] 孙骇浪[1] 冀园琦[1] 王瑞 杨溪洋 张天蕾 张嘉盈 YANGWei;GE Ming;SUN Hailang;JI Yuanqi;WANG Rui;YANGXiyang;ZHANGTianlei;ZHANG Jiaying(Department of Neurosurgery,Beijing Children′s Hospital,Captital Medical University,National Center for Children′s Health,No.56 Nanlishilu,Xicheng District,Beijing 100045,China)

机构地区：[1]首都医科大学附属北京儿童医院神经外科,国家儿童医学中心,北京100045

出　　处：《中国神经精神疾病杂志》2020年第7期385-389,共5页Chinese Journal of Nervous and Mental Diseases

摘　　要：目的总结小脑性缄默综合征患者的临床特征。方法采用病例对照设计,纳入2015~2019年间就诊于我院的后颅窝肿瘤患儿术后符合小脑性缄默综合征者为缄默组(n=41),未发生缄默者作为非缄默组(n=50),通过随访总结缄默的时间特点,并比较两组在肌张力、ECOG评分以及吞咽障碍方面的差异。结果与非缄默组比较,缄默组男性(80.5%)、肿瘤位于中线(78.0%)占比高,有统计学意义(P<0.05);年龄、病理类型(髓母细胞瘤)、肿瘤质地无显著差异(P>0.05)。缄默中位潜伏期为0(0,3)d,缄默中位持续时间为42(28,87)d。缄默组患儿术后ECOG评分[3(2,4)]、吞咽障碍[2(1,4)]较非缄默组[1(1,4),0(0,0)]高,肌张力评分[1(1,2)]较非缄默组[4(3,4)]低(P<0.05);缄默组患儿低张力持续中位时间为51(42,120)d;缄默持续时间和低张力持续时间正相关,强度中等(r=0.47,P<0.05),与患儿年龄、缄默潜伏期无统计学相关(P>0.05)。结论大多数缄默没有潜伏期。缄默患儿有更加显著的神经功能缺损,且缄默持续时间与运动障碍持续时间呈正相关,与年龄、缄默潜伏期无关。Objective To summarize the clinical features of cerebellar mutism syndrome.Methods A case-control design was adopted.The children with posterior cranial fossa tumor who met with cerebellar mutism syndrome from 2015 to 2019 were included in the CMS group(n=41),and those without were taken as the control group(n=50).The differences in dystonia,ECOG score and dysphagia between the two groups were compared based on the duration of mutism.Results Compared with the non-CMS group,male(80.5%)and tumor located at the midline(78.0%)accounted for a higher proportion(P<0.05)in CMS group,but there was no significant difference in age,pathological type(medulloblastoma)and tumor texture(P>0.05).The median latency of mutism was 0 d[0 d,3 d],and the median duration of mutism was 42 d[28 d,87 d].The postoperative ECOG score[3(2,4),dysphagia[2(1,4)]were significantly higher in the CMS group than in the control group[1(1,4),0(0,0)],while dystonia score[1(1,2)]was significantly lower in in the CMS group than in the control group[4(3,4)],with P<0.05.In the CMS group,the median duration of dystonia was 51 Most patients with mutism have no latent period.CMS children are often accompanied by more serious neurological impairment,and the duration of mutism is positively correlated with the duration of dyskinesia,but not age and latent period of mutsim.(42 d,120 d).The duration of mutism was positively correlated with the duration of dystonia(r=0.47,P<0.05)but not age and latent period of mutism.Conclusion Most patients with mutism have no latent period.CMS children are often accompanied by more serious neurological impairment,and the duration of mutism is positively correlated with the duration of dyskinesia,but not age and latent period of mutsim.

关 键 词：小脑性缄默综合征 临床特征 运动障碍 口咽障碍 

分 类 号：R726[医药卫生—儿科]