Lipid Deposition Myopathy Pathology And Histochemical Study

【Objective 】To explore accuratly,conveniently and practically the technology of skeletal muscle fibers distinguished of lipid storage myopathye and study the relation between skeletal muscle fibers lipid storage and clinic syndromes,we applied the technology of enzymatic cytochemistry.The trial is vital significence for dignosis and prognosis and a great supplement and development of traditional biopsy dignoss technology.【Methods 】muscles biopsy of 25 patients with lipid storage myopathy were freezed and cut into slices. Specimens by routine histology and acid or alkaly myosin ATPase, oil red O and PAS stain were observed the difference of types of keletal muscle fibers by ATPase stain and degree of fatty and glycogen dispersed in sarcoplasm,explored substancs metabolite .【Results 】There were a lot of fatty droplets in LSM patients' keletal muscle fibers;lipid storang of TypeI was more than that of TypeII;It was positive interrelated between the stornge of the fat in muscle fibers and clinical syndromes;Serum CK and LDH of LSM patients wereobviously higher than these kf nomal person (p<0.01);PAS reaction shomed glycogen capacity obviously descenged in LSM patients' muscle fibers,in which TypeIIA fibers were the most outstanding;M-ATPase showed there were difference among types of muscle fibers active,in which TypeI muscle fibers active were stronger than the others and TypeIIB muscle fibers active were weaker than the others.【Conclusion】We observed specimens that were stained by enzymatic cytochemistry , such as ATPase, ORO and PAS;meanwhile , we found that active of types of muscle fibers of LSM changed; fatty droplets added, which were positive interrelated with clinical syndromes; glycogen deserded; serum muscle enzyme raised. These characteristictics provided reliably consult index for LSM dignosis and distinguished dignosis with the other myopathy.