摘要
目的探讨MELAS综合征的临床特点及影像学特征。方法选取2011年1月~2018年5月经我院神经内科确诊的30例MELAS综合征患者的临床及影像学资料,对其临床资料、头颅CT、常规MRI、DWI、MRA、增强MRI、MRS及PWI进行回顾性分析。结果30例MELAS综合征患者中男性17例,女性13例,发病年龄5~58岁,中位年龄15岁,63.3%(19/30)以癫痫和卒中样发作为首发症状,36.0%(9/25)显示双侧基底节区对称性钙化,20.0%(5/25)CT扫描见片状低密度影,全部(30/30)MRI扫描见T1WI呈低信号,T2WI和FLAIR呈高信号,DWI呈高信号,ADC呈高信号或稍高信号,病灶主要分布于大脑皮层及皮层下白质,且88.9%(26/30)累及2个及2个以上脑叶,主要位于顶叶、枕叶和颞叶。1H-MRS发现MELAS综合征患者病灶NAA峰显著下降和Lac峰明显升高;增强MRI:29.4%(5/17)见线样或者脑回样强化,MRA:36.0%(9/16)见脑血管扩张、分支增多,PWI:77.8%(7/9)见rCBV和rCBF增加。结论MELAS综合征的临床及CT和MRI表现具有一定特征性,特别是MRI检查有助于本病的早期诊断。
Objective To study the clinical and imaging features of MELAS syndrome.Methods Clinical and imaging data of 30 patients with MELAS syndrome in the neurology department of our hospital from January 2011 to 2018 were collected.The clinical data,head CT,routine MRI,DWI,MRA,enhanced MRI,MRS and PWI were retrospectively analyzed.Results Of the 30 patients with MELAS syndrome,17 were males and 13 females,aged 5~58 years,with a median age of 15 years.63.3%(19/30)presented with epilepsy and apoplexy as initial symptoms,36.0%(9/25)showed symmetrical calcification in bilateral basal ganglia,20%(5/25)CT scans were scanned with low density shadow.All(30/30)MRI scan showed T1WI low signal,T2WI and FL AIR were high signal,DWI was high signal,ADC was high signal or slightly high signal.The lesions were mainly located in the cortex and subcortical white matter,and 88.9%(26/30)involved 2 and more than 2 cerebral lobes,mainly located in parietal lobe,occipital lobe and temporal lobe.1H-MRS found a significant decrease in the NAA peak of the MELAS syndrome and a significant increase in the Lac peak,and enhanced MRI showed 29.4%(5/17)was enhanced with linear or gyrus,MRA showed 36.0%(9/16)was found in cerebral vasodilatation,increased branches,and PWI showed 77.8%(7/9)increased in rCBV and rCBF.Conclusion The clinical manifestations,CT and MRI findings of MELAS syndrome are characteristic,especially that MRI examination is helpful to the early diagnosis of this disease.
作者
郭辉
程敬亮
阎晓朋
徐红卫
张敏
潘文玲
吴峰
GUO Hui;CHENG Jingliang;YAN Xiaopeng;XU Hongwei;ZHANG Min;PAN Wenling;WU Feng(Department of Radiology, The Fifth Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, P.R.China;Department of MRI, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, P.R.China;Department of Internal Neurology, The Fifth Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, P.R.China)
出处
《医学影像学杂志》
2021年第12期2010-2013,共4页
Journal of Medical Imaging