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目的分析脂质沉积性肌病(lipid storage myopathy,LSM)患者静息时、亚极限运动后及治疗前后股四头肌31磷-磁共振波谱成像(31P-MRS)表现,并探讨31P-MRS对LSM的诊断及疗效价值。资料与方法对经肌肉活检证实为LSM的9例患者行静息时、亚极限运动后即刻、5 min、10 min和15 min时31P-MRS成像。所有患者经强的松及左卡尼汀治疗后2～4个月(平均2.2个月)后再次行静息状态及亚极限运动后动态31P-MRS成像。对31P-MRS数据进行后处理,获得静息时磷酸肌酸(PCr)、无机磷(Pi)、Pi与三磷酸腺苷之比(Pi/ATP)、Pi/PCr、PCr/ATP、pH、二磷酸腺苷(ADP)、磷酸化能力(PP)和线粒体ATP生成率与线粒体最大产能量之比(Q/Vmax);运动后PCr生成率的速度常数(KPCr)、PCr最初生成率(ViPCr)和线粒体最大产能量(Vmax)等衡量线粒体功能的参数。并将9名年龄匹配的、未经专业体育训练的志愿者作为对照组与病变组静息时、亚极限运动后各项数据相对比。采用配对t检验比较分析病变组与对照组之间、病变组治疗前后的差别,所有数据用SPSS 13.0进行统计分析。结果 (1)治疗前病变组与对照组对比,将出现静息状态代谢物PCr、PCr/ATP、ADP静息状态指标1/PP及运动后指标ViPCr、KPCr、Vmax减小,经统计学分析差异有统计学意义。其他指标差异无统计学意义。(2)病变组治疗前后比较将出现静息状态代谢物ADP及运动后指标Vmax、ViPCr、KPCr减小,提示ADP及运动后参数更敏感。静息状态代谢物PCr、PCr/ATP及Pi、Pi/ATP无明显异常。结论31P-MRS通过运动前后多种参数判断肌肉能量代谢变化、线粒体功能及氧化PP,对LSM的诊断及疗效评估具有重要价值。
Objective To analyze the expression of 31P-MRS in the quadriceps femoris during rest, submaximal exercise and before and after treatment of lipid storage myopathy (LSM) Diagnosis and therapeutic value of LSM. Materials and Methods 31P-MRS imaging was performed on 9 patients who had LSM confirmed by muscle biopsy at rest, immediately after submaximal exercise, 5 min, 10 min and 15 min. All patients were treated with prednisone and levocarnitine for 2 to 4 months (mean, 2.2 months) after re-resting status and 31P-MRS imaging after submaximal exercise. The 31P-MRS data were processed to obtain the ratios of creatine phosphate (PCr), inorganic phosphate (Pi), Pi to adenosine triphosphate (Pi / ATP), Pi / PCr, PCr / ATP, pH, ADP, PP, and mitochondrial ATP production rate (Q / Vmax) of mitochondria; KPCr, ViPCr and mitochondria Maximum capacity (Vmax) and other parameters to measure mitochondrial function. Nine age-matched volunteers without professional training were used as control group to compare the data after submaximal exercise with the rest group. Paired t test was used to compare and analyze the difference between the lesion group and the control group before and after treatment. All the data were analyzed by SPSS 13.0. Results (1) Compared with the control group, resting state metabolites PCr, PCr / ATP, ADP resting state index 1 / PP and post-exercise indexes ViPCr, KPCr, Vmax decreased compared with the control group. The difference was statistically significant. No significant difference in other indicators. (2) Before and after treatment, ADP of resting state and Vmax, ViPCr and KPCr decreased after exercise, suggesting that ADP and post-exercise parameters are more sensitive. Resting metabolites PCr, PCr / ATP and Pi, Pi / ATP no significant abnormalities. Conclusion 31P-MRS can determine the changes of muscle energy metabolism, mitochondrial function and oxidized PP through various parameters before and after exercise, which is of great value in diagnosis and evaluation of LSM.