| Effect of fuels on exercise capacity in muscle phosphoglycerate mutase deficiency. | |
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MedLine Citation:
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PMID: 16157752 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Muscle phosphoglycerate mutase deficiency (PGAMD) is a rare, recessively inherited metabolic myopathy that affects one of the last steps of glycolysis. Clinically, PGAMD resembles muscle phosphorylase deficiency (McArdle disease) and phosphofructokinase deficiency (PFKD). However, it is unknown whether PGAMD is associated with a second-wind phenomenon during exercise, as in McArdle disease, and whether patients with PGAMD, like patients with PFKD and McArdle disease, benefit from supplementation with fuels that bypass the metabolic block. OBJECTIVE: To investigate whether fuels that bypass the metabolic block can improve exercise capacity or whether exercise capacity improves during sustained exercise. DESIGN: Single-blind, placebo-controlled investigation of the effects of glucose, lactate, and intralipid on work capacity in patients with PGAMD. SETTING: National University Hospital, University of Copenhagen, and Neuromuscular Center, Institute for Exercise and Environmental Medicine. Patients Two unrelated men (21 and 26 years old) with PGAMD who since their teens had experienced muscle cramps, muscle pain, and episodes of myoglobinuria provoked by brief vigorous exercise, 4 patients with McArdle disease (mean +/- SD age, 32 +/- 5 years) with 0% residual phosphorylase activity in muscle, and 6 healthy, untrained male volunteers (mean +/- SD age, 23 +/- 1 years) were studied. INTERVENTIONS: Using constant and variable workload protocols on a cycle ergometer, it was investigated whether a spontaneous second wind occurs during exercise in patients with PGAMD, and using a constant workload protocol followed by an incremental load to exhaustion, it was tested whether infusion of lactate, glucose, or intralipid alters the exercise tolerance in PGAMD. MAIN OUTCOME MEASURES: Whether a second wind occurs during exercise and whether fuels that bypass the metabolic block can improve exercise and oxidative capacity. RESULTS: In contrast to patients with McArdle disease, with whom they share many clinical features, in patients with PGAMD, cycle exercise and oxidative capacity are virtually normal, a second wind does not occur, and lipid and lactate supplements do not improve exercise capacity. CONCLUSION: Although the clinical manifestations of PGAMD mimic McArdle disease with respect to the presence of exertional muscle cramps, rhabdomyolysis, and myoglobinuria, this study shows that cycle exercise responses are strikingly different. |
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Authors:
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John Vissing; Bjørn Quistorff; Ronald G Haller |
Publication Detail:
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Type: Clinical Trial; Comparative Study; Controlled Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Archives of neurology Volume: 62 ISSN: 0003-9942 ISO Abbreviation: Arch. Neurol. Publication Date: 2005 Sep |
Date Detail:
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Created Date: 2005-09-13 Completed Date: 2005-11-14 Revised Date: 2009-11-03 |
Medline Journal Info:
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Nlm Unique ID: 0372436 Medline TA: Arch Neurol Country: United States |
Other Details:
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Languages: eng Pagination: 1440-3 Citation Subset: AIM; IM |
Affiliation:
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Copenhagen Muscle Research Center, National University Hospital, Denmark. vissing@rh.dk |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Exercise* Exercise Test / methods Glucose / administration & dosage* Glycogen Phosphorylase, Muscle Form / deficiency Heart Rate / drug effects, physiology Humans Lactase / administration & dosage* Magnetic Resonance Spectroscopy / methods Male Muscle, Skeletal / drug effects*, enzymology, pathology Phosphoglycerate Mutase / deficiency* Single-Blind Method Time Factors Treatment Outcome |
| Chemical | |
Reg. No./Substance:
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50-99-7/Glucose; EC 2.4.1.-/Glycogen Phosphorylase, Muscle Form; EC 3.2.1.108/Lactase; EC 5.4.2.1/Phosphoglycerate Mutase |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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