title: 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.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Japan.
authors.forename: Kiyomi
authors.initials: K
authors.lastname: Komura
authors.forename: Elke
authors.initials: E
authors.lastname: Hobbiebrunken
authors.forename: Ekkehard K G
authors.initials: EK
authors.lastname: Wilichowski
authors.forename: Folker A
authors.initials: FA
authors.lastname: Hanefeld
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003401:Creatine / Q000008:administration & dosage* / Q000009:adverse effects; D005080:Exercise Test; D005260:Female; D006801:Humans; D019344:Lactic Acid / Q000097:blood; D017241:MELAS Syndrome / Q000175:diagnosis / Q000188:drug therapy; D008297:Male; D017237:Mitochondrial Encephalomyopathies / Q000175:diagnosis / Q000188:drug therapy*; D009043:Motor Activity / Q000187:drug effects; D017060:Patient Satisfaction; D012189:Retrospective Studies; D012420:Running; D014797:Vital Capacity / Q000187:drug effects
chemical_list: D019344:Lactic Acid; D003401:Creatine