Effectiveness of creatine monohydrate in mitochondrial encephalomyopathies.

Abstract

The mitochondrial encephalomyopathies are chronic progressive disorders affecting predominantly the neuromuscular system. Symptoms are induced by insufficient energy supply resulting from a deficiency of oxidative phosphorylation. We studied one male and four female patients with genetically proven mitochondrial encephalomyopathy. Their ages ranged from 7 to 19 years (two with Kearns-Sayre syndrome, one patient with neuronal muscle weakness, ataxia, and retinitis pigmentosa syndrome, and two patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes), using a retrospective study method. We studied the effect of creatine supplementation (0.08 g-0.35 g/kg body weight/day; 9 months to 4 years, 10 months) and measured skeletal muscle power analysis (bicycle ergometer). After creatine supplementation all patients demonstrated an increase in their maximum performance (W) (+4% - +30%; mean: +12.1%). These results indicate an improved aerobic oxidative function of mitochondria after creatine administration in patients with mitochondrial encephalomyopathies. Continuous physical exercise was improved to a greater extent than instantaneous activity.

• Authors: Kiyomi Komura, Elke Hobbiebrunken, Ekkehard K G Wilichowski, Folker A Hanefeld
• Journal: Pediatric neurology (Pediatr Neurol) Vol. 28 Issue 1 Pg. 53-8 (Jan 2003) ISSN: 0887-8994 [Print] United States
• PMID: 12657421 (Publication Type: Journal Article)

Chemical References

• Lactic Acid
• Creatine

Topics

• Adolescent
• Adult
• Child
• Creatine (administration & dosage, adverse effects)
• Exercise Test
• Female
• Humans
• Lactic Acid (blood)
• MELAS Syndrome (diagnosis, drug therapy)
• Male
• Mitochondrial Encephalomyopathies (diagnosis, drug therapy)
• Motor Activity (drug effects)
• Patient Satisfaction
• Retrospective Studies
• Running
• Vital Capacity (drug effects)