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目的:通过研究脊髓小脑共济失调3型(SCA3/MJD)患者小脑蚓部、桥臂、小脑皮质、齿状核的氢质子磁共振波谱(1H-MRS)的特点,探讨1H-MRS评估SCA3型疾病严重程度的价值。方法:对36例SCA3/MJD患者与27例性别、年龄与之匹配的健康对照进行单体素1H-MRS测定,比较2组间小脑上述各脑区N-乙酰天门冬氨酸(NAA)/肌酸(Cr),胆碱(Cho)/Cr,NAA/Cho比值的差别;同时对34例患者进行MJD1基因中(CAG)n重复次数的测定,应用共济失调等级量表(SARA)对其进行评分,并结合上述各脑区代谢物比值进行相关性分析。结果:SCA3/MJD患者的SARA评分与其病程、发病年龄、CAG重复次数呈正相关,与发病年龄呈负相关;SCA3/MJD患者上述各脑区NAA/Cr值及齿状核、蚓部NAA/Cho值较正常对照明显降低(P<0.01);SCA3/MJD患者小脑各区的1H-MRS与病程、发病年龄、CAG重复次数、SARA评分存在相关性。结论:1H-MRS技术可以检测SCA3/MJD患者小脑的神经代谢改变,且可较好地反应SCA3/MJD的疾病严重程度。
Objective: To investigate the characteristics of proton magnetic resonance spectroscopy (1H-MRS) in the cerebellar vermis, brachiocephalic, cerebellar cortex and dentate gyrus in patients with spinocerebellar ataxia type 3 (SCA3 / MJD) The value of disease severity. Methods: Monomer 1H-MRS was performed in 36 SCA3 / MJD patients and 27 healthy controls with the same gender and age. The levels of N-acetyl-aspartate (NAA) Creatinine (Cr), choline (Cho) / Cr, NAA / Cho ratio of 34 cases of patients with MJD1 gene (CAG) n repetition number determination, the application of ataxia rating scale (SARA) It was scored and combined with the ratio of metabolites in each brain area for correlation analysis. Results: The SARA score of patients with SCA3 / MJD was positively correlated with the course of disease, the age of onset and the number of CAG repeat, and was negatively correlated with the age of onset. The NAA / Cr values of these brain regions and the ratio of NAA / Cho (P <0.01). 1H-MRS in the cerebellum of patients with SCA3 / MJD correlated with duration of disease, age of onset, number of CAG repeat and SARA score. Conclusion: 1H-MRS can detect the changes of cerebellar neuro-metabolism in patients with SCA3 / MJD and can well reflect the severity of SCA3 / MJD disease.