The Series Study In Magnetic Resonance Imaging Of SCA3/MJD

ChapterⅠ1H MR spectroscopy study of SCA3/MJDOBJECT:This study focused on metabolic alterations of several regions in cerebellum and putamen and thalamus in SCA3/MJD patients, also correlated with the ataxia rating scale and illness duration.METHOD:Thirty-one SCA3/MJD patients and 31 sex- and age-matched healthy control subjects were studied at 1.5T scanner,using point resolved spectroscopy(TR,1500 ms;TE,135 ms).Firstly,we determined NAA/Cr,Cho/Cr,and NAA/Cho in the middle cerebellar peduncle,dentate,cerebellar vermis,cerebellar cortex,putamen,and thalamus.Secondly,the difference of NAA/Cr,Cho/Cr,and NAA/Cho in these regions between the two groups was determined,followed by the difference of NAA/Cr,Cho/Cr,and NAA/Cho between the left and right side in each group.Finally,we correlated NAA/Cr,Cho/Cr,and NAA/Cho with ICARS and SARA and illness duration in SCA3/MJD patients group.RESULT:In the patients group,we found that NAA/Cr,and NAA/Cho in the middle cerebellar peduncle,dentate,cerebellar vermis, and thalamus were significantly below normal(P＜0.01),whereas only NAA/Cr in cerebellar cortex was significantly below(P＜0.01).Cho/Cr differences in cerebellar vermis was significantly below normal(P＜0.01),whereas only showed a trend toward below-normal values in dentate(P=0.06).The total ICARS and SARA scores and subscores were negatively correlated with NAA/Cr in the middle cerebellar peduncle and dentate in three domains of upper limb ataxia,ataxia of posture and gait and dysarthria(r=-0.40～-0.70,P＜0.05 or 0.01).The duration years of illness duration was negatively correlated with NAA/Cr in the middle cerebellar peduncle and dentate(r=-0.50～-0.60,P＜0.01).Similarly, the total ICARS and SARA scores and subscores were negatively correlated with Cho/Cr in the dentate in the same three domains(r=-0.40～-0.50,P＜0.05 or 0.01).The duration years of illness duration was negatively correlated with Cho/Cr in the dentate(r=-0.51,P＜0.01)CONCLUSION:Including cerebellar vermis,cerebellar cortex,and thalamus,SCA3/MJD mainly affected the middle cerebellar peduncle and dentate,where the degree of neuronal dysfunction accompanied by comparable cerebellar ataxia and illness duration. ChapterⅡResearch of Diffusion tensor imaging in SCA3/MJDOBJECT:This study focused on the white-matter integrity of the whole brain in SCA3/MJD patients,also correlated the white-matter integrity of cerebellum with the ataxia rating scale and illness duration.METHOD:Twenty-nine SCA3/MJD patients and 30 sex- and age-matched healthy control subjects were studied with single shot SEEPI. Firstly,fractional anisotropy(FA)values of the white matter of the whole brain between the two groups was measured with an automated voxel-based method of analysis.Secondly,fractional anisotropy and mean diffusivity(MD)values of all three cerebellar peduncles of the two groups were measured with the trractography barfed group mapping. Finally,FA and MD values of all three cerebellar peduncles were correlated with ICARS and SARA and illness duration in patientsgroup.RESULT:There was reduced FA value in SCA3/MJD patients in white matter of the cerebellum and brainstem(p＜0.0001).There were statistically significant decrease in FA and volume and increase in MD between SCA3/MJD patients and the control subjects in all three. cerebellar peduncles(p＜0.0001).The total ICARS and SARA scores together with subscores were negatively correlated with FA in all three cerebellar peduncles in three domains of limb ataxia,ataxia of posture and gait,and dysarthria.(r=-0.40～-0.70,P＜0.05 or 0.01).However, the total ICARS and SARA scores together with subscores were negatively correlated with MD only in the super-cerebellar peduncle in the same three domains(r=-0.40～-0.60,P＜0.05 or 0.01).Similarly, the duration years of illness duration was negatively correlated with FA in all three cerebellar peduncles(r=-0.50～-0.70,p＜0.01),and correlated with MD in the super-cerebellar peduncle only(r=0.63,p＜0.01)CONCLUSION:The tracts in cerebellum and brainstem were mainly affected in SCA3/MJD.There were significant atrophy and demyelinization and axonal loss in all three cerebellar peduncles in SCA3/MJD.The finding of axonal loss in all three cerebellar peduncles were correlated with cerebellar ataxia and illness duration.Beside MCP and ICP,the affection of SCP was most important in SCA3/MJD patients. ChapterⅢResearch of Voxel-based morphometry in SCA3/MJDOBJECT:This study focused on the volumetric changes of white matter and gray matter of the whole brain in SCA3/MJD patients,also correlated with the ataxia rating scale and illness duration.METHOD:Thirty SCA3/MJD patients and 30 sex- and agematched healthy control subjects were studied with 3D MPRAGE.We used optimized voxel-based morphometry based on high-resolution T1-weighted MRI to compare gray and white matter volume between the two groups.Additionally,a correlation between the atrophic regions and the ICARS and SARA and illness duration was performed using a multiple regression algorithm.RESULT:We observed significant gray matter volume loss in the cerebellar hemispheres(excludingⅡ,Ⅲlobule),cerebellar vermis(includingⅣ,Ⅴlobule),brainstem,thalamus and temporal lobes (p=0.00001).Significant white matter volume loss was observed in the cerebellar hemispheres,brainstem,frontal lobes,and corpus callosum in the right side,temporal in the left side(p=0.00001).The highest degree of gray matter atrophy in the cerebellar tonsil(in the left side)was correlated with the total ICARS and SARA score and illness duration(p =0.01)(p=0.05).The white matter atrophy in the cerebellum and brainstem were correlated with the ICARS and SARA score and illness duration(p=0.001)(p=0.005)(p=0.01).CONCLUSION:SCA3/MJD represents a form of brainstemcerebellar atrophy with affection of cerebrum.Significant gray matter volume loss in the cerebellar hemispheres(excludingⅡ,Ⅲlobule), midbrain,medulla,thalamus and temporal lobes was first reported. Significant white matter volume loss in the midbrain,medulla,frontal lobes,and corpus callosum in the right side,temporal in the left side was also first reported.The gray matter atrophy in the cerebellar tonsil(in the left side)was correlated with the cerebellar ataxia and illness duration. The white matter atrophy in the cerebellum and brainstem were correlated with cerebellar ataxia and illness duration...