| Blocked muscle fat oxidation during exercise in neutral lipid storage disease. | |
MedLine Citation:
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PMID: 22491199 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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OBJECTIVE: To determine whether impaired exercise capacity in neutral lipid storage disease with myopathy is solely caused by muscle weakness or whether a defect in energy metabolism (blocked fat oxidation) may also play a role. DESIGN: We studied a 37-year-old woman with neutral lipid storage disease with myopathy, who cycled while lipid oxidation was assessed using U-(13)C palmitate tracer dilution technique. The effect of a glucose infusion during exercise was also studied. SETTING: Neuromuscular research unit. RESULTS: The exercise-induced increase in fat oxidation was virtually abolished in the patient. Treatment with intravenous glucose infusion improved maximal oxygen uptake from 23 to 27 mL × kg(-1) × min(-1), and maximal workload from 75 to 100 W. CONCLUSIONS: These results demonstrate that in addition to fixed weakness, neutral lipid storage disease with myopathy is also characterized by a profound block in fat oxidation, which limits exercise tolerance. |
Authors:
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Pascal Laforêt; Mette Orngreen; Nicolai Preisler; Grete Andersen; John Vissing |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Archives of neurology Volume: 69 ISSN: 1538-3687 ISO Abbreviation: Arch. Neurol. Publication Date: 2012 Apr |
Date Detail:
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Created Date: 2012-04-11 Completed Date: - Revised Date: - |
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: 530-3 Citation Subset: AIM; IM |
Affiliation:
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Centre de Référence de Pathologie Neuromusculaire Paris-Est, Bâtiment Babinski, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75651 Paris CEDEX 13, France. pascal.laforet@psl.aphp.fr. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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