The Clinical Value Of ³¹P-MRS In Patients With Lipid Storage Myopathy

Objective:To investigate the 31P magnetic resonance spectroscopy (31P-MRS) manifestations of skeletal muscle in patients with lipid storage myopathy (LSM) and evaluate its clinical value in auxiliary diagnosis and therapeutic effect.Methods:31P-MRS scanning was performed in 12 patients prior to and after treatment and 11 collators. The areas under resonance of inorganic phosphate (Pi), phosphocreatine (PCr) and adenosine triphosphate (ATP) were calculated from the 31P-MRS images, then the ratios of Pi/ATP, PCr/ATP and Pi/PCr and the values of Pi, PCr, intracellular PH (PHint), adenosine diphosphate (ADP) and phosphorylation potential (PP) were calculated at last. The above-mentioned variables were compared among LSM patients prior to treatment and the control group, and variables were also compared in LSM patients prior to and after treatment.Results:Compared with the control group, LSM patients prior to treatment had significantly lower PCr, PCr/ATP and PP (P<0.05), and significantly higher Pi/PCr and ADP (P<0.05), but there were no significant differences in Pi, Pi/ATP and PHint between the control group and LSM patients prior to treatment (P>0.05). Compared with LSM patients prior to treatment, LSM patients after treatment had significantly higher PCr, PCr/ATP and PP (P<0.05), and significantly lower ADP (P<0.05), but there were no significant differences in Pi, Pi/ATP, Pi/PCr and PHint between LSM patients prior to and after treatment (P>0.05).Conclusion:31P-MRS can monitor the variations of muscle energetic metabolism in LSM patients noninvasively, which is useful for the auxiliary diagnosis and can be applied to assess the treatment response.