Diagnosis and treatment of lipid deposition myopathy

  Lipid deposition myopathy is a metabolic myopathy. Mainly the deficiency of various enzymes fatty acid oxidation disorders in myocyte mitochondria, resulting in: 1, difficulties in energy supply; 2, large accumulation of lipid droplets destroying myocyte structure and function; 3, due to the different enzymes causing fatty acid oxidation disorders, the corresponding clinical manifestations produced also differ; 4, primary carnitine deficiency myopathy; 5, carnitine palmitoyltransferase deficiency; 6, lipid acyl coenzyme a dehydrogenase deficiency; 7 7. carnitine carnitine translocase deficiency; 8. triglyceride accumulation disease with impaired oxidation of long-chain fatty acids.  Treatment: There is no cure, but certain types can relieve symptoms.  Levocarnitine: For primary carnitine deficiency myopathy, carnitine supplementation. [Take with meals. Adults 1g daily in 2-3 doses; children start at 50mg per kg body weight and slowly increase the dose as needed and tolerated, usually 50-100mg per kg body weight (maximum dose not to exceed 3g a day)]. Long-term use is required.  Riboflavin (vitamin b2): for riboflavin-responsive lipid deposition myopathy. 100mg to 200mg tid. High-fat diet should be avoided, high-carbohydrate diet promoted, exercise should be appropriate, avoid strenuous exercise.