Treatment of mitochondrial myopathy due to complex III deficiency with vitamins K3 and C: A 31P-NMR follow-up study.
A patient with mitochondrial myopathy due to complex III deficiency who was treated with vitamin K3 (menadiol sodium diphosphate, 40 mg daily) and vitamin C showed clinical improvement. A 1-year study with phosphorus 31 nuclear magnetic resonance (31P-NMR) monitoring has shown that clinical and metabolic improvement was maintained by this therapy; increasing the dose of vitamin K3 to 80 mg daily improved the bioenergetic state of the patient's muscles at rest; postexercise recovery was less responsive to the increased dose; and a higher dose of vitamin K3 (80 mg/day) did not produce side effects. The differential therapeutic effects of vitamin K3 at rest and during exercise recovery are probably due to the differential kinetics of each metabolic state. Monitoring muscle bioenergetics with 31P-NMR is valuable in documenting therapeutic improvements in mitochondrial myopathies.
D000328:Adult; D001205:Ascorbic Acid / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D014450:Electron Transport Complex III; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D009097:Multienzyme Complexes / Q000172:deficiency*; D009132:Muscles / Q000378:metabolism*; D009135:Muscular Diseases / Q000188:drug therapy* / Q000378:metabolism; D010710:Phosphates / Q000378:metabolism; D010725:Phosphocreatine / Q000378:metabolism; D011808:Quinone Reductases / Q000172:deficiency*; D014812:Vitamin K / Q000627:therapeutic use*
D009097:Multienzyme Complexes; D010710:Phosphates; D010725:Phosphocreatine; D014812:Vitamin K; D011808:Quinone Reductases; D014450:Electron Transport Complex III; D001205:Ascorbic Acid