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Blocked muscle fat oxidation during exercise in neutral lipid storage disease.
MedLine Citation:
PMID:  22491199     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
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:
Pascal Laforêt; Mette Orngreen; Nicolai Preisler; Grete Andersen; John Vissing
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Archives of neurology     Volume:  69     ISSN:  1538-3687     ISO Abbreviation:  Arch. Neurol.     Publication Date:  2012 Apr 
Date Detail:
Created Date:  2012-04-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  530-3     Citation Subset:  AIM; IM    
Affiliation:
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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