Blocked muscle fat oxidation during exercise in neutral lipid storage disease

Pascal Laforêt; Mette Ørngreen; Nicolai Preisler; Grete Andersen; John Vissing

doi:10.1001/archneurol.2011.631

Blocked muscle fat oxidation during exercise in neutral lipid storage disease

Pascal Laforêt, Mette Ørngreen, Nicolai Preisler, Grete Andersen, John Vissing

23 Citationer (Scopus)

Abstract

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-13C 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 27mL × 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.

Originalsprog	Engelsk
Tidsskrift	Archives of Neurology
Vol/bind	69
Udgave nummer	4
Sider (fra-til)	530-3
Antal sider	4
ISSN	0003-9942
DOI	https://doi.org/10.1001/archneurol.2011.631
Status	Udgivet - apr. 2012

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10.1001/archneurol.2011.631

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title = "Blocked muscle fat oxidation during exercise in neutral lipid storage disease",

abstract = "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-13C 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 27mL × 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.",

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T1 - Blocked muscle fat oxidation during exercise in neutral lipid storage disease

AU - Laforêt, Pascal

AU - Ørngreen, Mette

AU - Preisler, Nicolai

AU - Andersen, Grete

AU - Vissing, John

PY - 2012/4

Y1 - 2012/4

N2 - 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-13C 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 27mL × 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.

AB - 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-13C 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 27mL × 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.

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DO - 10.1001/archneurol.2011.631

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Blocked muscle fat oxidation during exercise in neutral lipid storage disease

Abstract

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