[1]000 武汉,华中科技大学同济医学院附属同济医院儿童神经内科[杨清梅 陈丽卿 刘艳(通信作者)],神经内科(薛峥).POLG突变引起的线粒体神经胃肠型脑肌病样综合征的临床特征[J].卒中与神经疾病杂志,2023,30(04):402-405,408.[doi:10.3969/j.issn.1007-0478.2023.04.015]
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POLG突变引起的线粒体神经胃肠型脑肌病样综合征的临床特征()
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《卒中与神经疾病》杂志[ISSN:1007-0478/CN:42-1402/R]

卷:
第30卷
期数:
2023年04期
页码:
402-405,408
栏目:
论著
出版日期:
2023-08-20

文章信息/Info

文章编号:
1007-0478(2023)04-0402-05
作者:
430030 武汉华中科技大学同济医学院附属同济医院儿童神经内科[杨清梅 陈丽卿 刘艳(通信作者)]神经内科(薛峥)
杨清梅 陈丽卿 薛峥 刘艳
关键词:
线粒体聚合酶γ基因 线粒体DNA 线粒体神经胃肠型脑肌病 线粒体神经胃肠型脑肌病样综合征
分类号:
R742
DOI:
10.3969/j.issn.1007-0478.2023.04.015
文献标志码:
A
摘要:
目的 探讨线粒体聚合酶γ基因(Polymerase gamma,POLG)突变引起的线粒体神经胃肠型脑肌病(Mitochondrial neurogastrointestinal encephalomyopathy,MNGIE)样综合征患者的临床特征。方法 报道1例POLG突变引起的MNGIE样综合征患者的临床资料,并检索建库至2022年9月的中国知网、万方中文数据库和PubMed数据库中关于POLG突变引起的MNGIE样综合征的病例,从而总结该疾病的临床特点。结果 本例患者为6月龄女婴,以胃肠道症状及运动发育落后为主要表现,脑脊液蛋白水平升高,肌电图示周围神经损害,高通量测序分析发现POLG基因2个杂合突变点,分别为c.2591A>G和c.2962G>C。回顾文献,本研究共纳入11例(含本例)POLG突变引起的MNGIE样综合征患者; 起病年龄为3月龄-42岁,临床症状包括胃肠道症状(11例)、周围神经损害(11例)、上睑下垂(9例)、肌无力或运动不耐受(9例)、运动发育落后(2例)、共济失调(4例)、视力障碍(3例)、听力障碍(2例)、认知障碍或智力低下(2例)、癫痫发作(1例)、偏头痛(1例)及性功能减退(1例)。胃肠影像学检查存在胃肠道动力障碍(7/8)。肌电图为神经源性损害(10/10)或合并肌源性损害(3/10)。头部磁共振(Magnetic resonance imaging,MRI)显示白质脑病改变(3/10)。血乳酸水平升高(5/8),脑脊液蛋白水平升高(4/4)。肌肉活检(6/6)均支持线粒体肌病改变。POLG突变位点包括c.752C>T、c.1760C>T、c.2591A>T、c.895A>C、c.2243G>C、c.2857C>T、c.1399G>A、c.2669C>A、c.895A>C、c.3626_3629dupGATA、c.3643+1G>A、c.2396C>A、c.2391G>T、c.2962G>C、c.2591A>G。结论 POLG突变引起的MNGIE样综合征除具有经典MNGIE的临床特点外,还可具有POLG综合征的部分临床特征; 脑脊液检查可提供诊断线索,基因检测仍是目前主要的诊断手段。

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更新日期/Last Update: 2023-08-20