Clinical And Pathological Studies Of Lipid Storage Myopathy

Objective(1) To evaluate the clinical features and the therapy effects of lipid storage myopathy (LSM).(2) To investigate the pathological characteristics of lipid storage myopathy.The etiopathogenisis and differential diagnosis are probed into.Methods(1)Using retrospective analysis, the clinical manifestation, Laboratory Features and therapeutic methods of 18 LSM cases were analyzed.(2)Eighteen cases of lipid storage myopathy diagnosed by muscle biopsies with microscopic and electronmicroscopic examination are analyzed.Quadriceps or gastrocnemius were biopsied.Muscles amples were stained with routine histology and inspected by microscopy.Thin sections were stained with uranyl acetate followed by lead citrate prior to examination in a electronmicroscopy.Results(1) LSM was due to disorders of fatty acid oxidation.Symptoms were characterized by fatigue and muscle weakness, Proximal limb weakness was commom in 94.4% of the cases. Gastrointestinal symptoms were found in 33.3%.Increase of muscle enzymes were detected in 88.9% of all the patients, especially the serum creatine kinase (CK) lever.The myogenic lesions of electromyogram abnormality was seen in 83.3%.Steroids and vitamin B were effective in 83.3% of the patients.(2) Vacuole or crack of muscular fibers involved most of the patients. Electron microscopy shows the lipid vacuoles are not membrane-bound and are located in rows between typeâ… myofibrils.ConclusionThe pathogeny of lipid storage myopathy is varied.The confirmed diagnosisis depend on pathological features of muscle biopsy.Treatment with corticoid,carnitine,vitamins and food containing carnitine rich is effective.It should be select the special treatment method if the pathogeny is clear.