1H-MRS对SCA3/MJD的诊断价值及与临床病程的关系

摘要

目的:探讨氢质子波谱(proton magnetic resonance spectroscopy,1H-MRS)在脊髓小脑型共济失调3型/马查多-约瑟夫病(hereditary spinocerebellar ataxias 3 and Machado-Joseph,SCA3/MJD)诊断中的价值,同时探讨其与临床病程评分之间的联系.方法:SCA3/MJD起病患者、SCA3/MJD未起病患者及年龄匹配健康志愿者进行1H-MRS检查,经数据后处理获得N-乙酰天门冬氨酸(N-acetylaspartate,NAA),肌酸(creatine,Cr),胆碱(choline-containing compounds,Cho),肌醇(myo-inositol,mI)值及NAA/Cr,Cho/Cr,mI/Cr比值.将以上数据进行对比研究;同时对于SCA3/MJD起病患者进行国际协作共济失调评估量表(international Cooperative Ataxia Rating Scale,ICARS)及共济失调等级量表(Scalefor the Assessment and Rating of Ataxia,SARA)评估,计算NAA/Cr,Cho/Cr,mI/Cr比值与临床评分之间的相关性.结果:SCA3/MJD起病患者在桥脑及齿状核的NAA/Cr值较正常对照组明显减低.SCA3/MJD起病患者小脑齿状核NAA/Cr值与ICARS评分有明显相关性.结论:SCA3/MJD的病变主要位于小脑及脑干,ICARS评分与小脑病变的程度有相关性,能较好地反映临床表现的轻重程度.1H-MRS对于SCA3/MJD的诊断有一定的应用前景.%Objective:To investigate the value of proton magnetic resonance spectroscopy (1H-MRS) on the diagnosis of SCA3/MJD,and to calculate the correlation between 1H-MRS ratio and the clinical score.Methods:Sixteen patients with SCA3/MJD and 19 healthy volunteers were scanned with 1H-MRS.The data of N-acetyl aspartate,creatine,choline-containing compounds,myoinositol,NAA/Cr,Cho/Cr,and mI/Cr ratio were collected,which were grouped for comparative study.The onset patients with SCA3/MJD were evaluated with the International Cooperative Ataxia Rating Scale and Scale for the Assessment and Rating of Ataxia,the correlation between NAA/Cr,Cho/Cr or mI/Cr ratio and the clinical score was calculated.Results:The NAA/Cr in the pons and cerebellar dentate nucleus from the onset patients with SCA3/MJD was significantly reduced compared to that in the normal control group.The NAA/Cr in the cerebellar dentate nucleus of onset patients with SCA3/MJD was obviously correlated with ICARS.Conclusion:SCA3/MJD lesions are mainly located in the cerebellum and brainstem,where gray and white mater are also involved.The cerebellar dentate nucleus may be the earliest involved area.There is a correlation between the ICARS and the cerebellar lesion degree.The ICARS reflects the severity of clinical manifestations.1H-MRS is useful in the diagnosis of SCA3/MJD.