title: [Value of 1H-MRS on SCA3/MJD diagnosis and clinical course].
abstract: 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.
authors.affiliation: Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, China.
authors.forename: Wu
authors.initials: W
authors.lastname: Xing
authors.affiliation: Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, China.
authors.forename: Xinxin
authors.initials: X
authors.lastname: Liao
authors.affiliation: Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, China.
authors.forename: Tingting
authors.initials: T
authors.lastname: Guan
authors.affiliation: Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, China.
authors.forename: Fangfang
authors.initials: F
authors.lastname: Xie
authors.affiliation: Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, China.
authors.forename: Lu
authors.initials: L
authors.lastname: Shen
authors.affiliation: Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, China.
authors.forename: Weihua
authors.initials: W
authors.lastname: Liao
authors.affiliation: Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, China.
authors.forename: Xiaoyi
authors.initials: X
authors.lastname: Wang
mesh_terms: D001224:Aspartic Acid / Q000032:analysis; D001933:Brain Stem / Q000737:chemistry / Q000000981:diagnostic imaging; D016022:Case-Control Studies; D002529:Cerebellar Nuclei / Q000737:chemistry / Q000000981:diagnostic imaging; D002531:Cerebellum / Q000737:chemistry / Q000000981:diagnostic imaging; D002794:Choline / Q000032:analysis; D003401:Creatine / Q000032:analysis; D006801:Humans; D017827:Machado-Joseph Disease / Q000000981:diagnostic imaging* / Q000378:metabolism; D009682:Magnetic Resonance Spectroscopy; D011149:Pons / Q000737:chemistry / Q000000981:diagnostic imaging; D066244:Proton Magnetic Resonance Spectroscopy
chemical_list: D001224:Aspartic Acid; D003401:Creatine; D002794:Choline