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Published in: European Journal of Applied Physiology 5/2010

01-11-2010 | Original Article

Excessive skeletal muscle recruitment during strenuous exercise in McArdle patients

Authors: Dale E. Rae, Timothy D. Noakes, Alejandro F. San Juan, Margarita Pérez, Gisela Nogales-Gadea, Jonatan R. Ruiz, María Morán, Miguel A. Martín, Antoni L. Andreu, Joaquín Arenas, Alejandro Lucia

Published in: European Journal of Applied Physiology | Issue 5/2010

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Abstract

We compared the cardiorespiratory response and muscle recruitment [as determined by electromyography (EMG)] of 37 McArdle patients [19 males, 37.4 ± 2.8 years, body mass index (BMI): 25.1 ± 4.7 kg m−2] and 33 healthy controls (18 males, 36.4 ± 10.0 years, BMI: 25.7 ± 3.8 kg m−2) during cycle-ergometer exercise (an incremental test to exhaustion and a 12-min submaximal constant workload test). We obtained cardiorespiratory [oxygen uptake and heart rate (HR)] and EMG data (rectus femoris and vastus lateralis muscles). During the incremental test, the patients exhibited the expected hyperkinetic cardiovascular response shown by a marked increase in the slope of the HR:Power relationship (p < 0.001). Throughout the incremental test and at the point of fatigue, the patients produced significantly less power than the controls (peak power output: 67 ± 21 vs. 214 ± 56 watts respectively, p < 0.001), yet they demonstrated significantly higher levels of muscle activity for a given absolute power. During the constant workload test, patients displayed higher levels of EMG activity than the controls during the second half of the test, despite a lower power production (34 ± 13 vs. 94 ± 29 watts respectively, p < 0.001). In conclusion, since the McArdle patients required more motor unit recruitment for a given power output, our data suggest that the state of contractility of their muscles is reduced compared with healthy people. Excessive muscle recruitment for a given load could be one of the mechanisms explaining the exercise intolerance of these patients.
Literature
go back to reference Allen DG, Lamb GD, Westerblad H (2008) Skeletal muscle fatigue: cellular mechanisms. Physiol Rev 88:287–332CrossRefPubMed Allen DG, Lamb GD, Westerblad H (2008) Skeletal muscle fatigue: cellular mechanisms. Physiol Rev 88:287–332CrossRefPubMed
go back to reference Borg GA (1982) Psychophysical bases of physical exertion. Med Sci Sports Exerc 14:377–381PubMed Borg GA (1982) Psychophysical bases of physical exertion. Med Sci Sports Exerc 14:377–381PubMed
go back to reference Brooks GA, Butterfield GE, Wolfe RR, Groves BM, Mazzeo RS, Sutton JR, Wolfel EE, Reeves JT (1991) Increased dependence on blood glucose after acclimatization to 4, 300 m. J Appl Physiol 70:919–927CrossRefPubMed Brooks GA, Butterfield GE, Wolfe RR, Groves BM, Mazzeo RS, Sutton JR, Wolfel EE, Reeves JT (1991) Increased dependence on blood glucose after acclimatization to 4, 300 m. J Appl Physiol 70:919–927CrossRefPubMed
go back to reference Chavarren J, Calbet JA (1999) Cycling efficiency and pedalling frequency in road cyclists. Eur J Appl Physiol Occup Physiol 80:555–563CrossRefPubMed Chavarren J, Calbet JA (1999) Cycling efficiency and pedalling frequency in road cyclists. Eur J Appl Physiol Occup Physiol 80:555–563CrossRefPubMed
go back to reference Di Mauro S (2007) Muscle glycogenoses: an overview. Acta Myol 26:35–41PubMed Di Mauro S (2007) Muscle glycogenoses: an overview. Acta Myol 26:35–41PubMed
go back to reference Dirksen RT (2009) Sarcoplasmic reticulum-mitochondrial through-space coupling in skeletal muscle. Appl Physiol Nutr Metab 34:389–395CrossRefPubMed Dirksen RT (2009) Sarcoplasmic reticulum-mitochondrial through-space coupling in skeletal muscle. Appl Physiol Nutr Metab 34:389–395CrossRefPubMed
go back to reference Farina D, Merletti R, Enoka RM (2004) The extraction of neural strategies from the surface EMG. J Appl Physiol 96:1486–1495CrossRefPubMed Farina D, Merletti R, Enoka RM (2004) The extraction of neural strategies from the surface EMG. J Appl Physiol 96:1486–1495CrossRefPubMed
go back to reference Gonzalez-Freire M, Santiago C, Gomez-Gallego F, Perez M, Foster C, Arenas J, Lucia A (2009) Does the K153R variant of the myostatin gene influence the clinical presentation of women with McArdle disease? Neuromuscul Disord 19:220–222CrossRefPubMed Gonzalez-Freire M, Santiago C, Gomez-Gallego F, Perez M, Foster C, Arenas J, Lucia A (2009) Does the K153R variant of the myostatin gene influence the clinical presentation of women with McArdle disease? Neuromuscul Disord 19:220–222CrossRefPubMed
go back to reference Haller RG, Lewis SF, Cook JD, Blomqvist CG (1983) Hyperkinetic circulation during exercise in neuromuscular disease. Neurology 33:1283–1287PubMed Haller RG, Lewis SF, Cook JD, Blomqvist CG (1983) Hyperkinetic circulation during exercise in neuromuscular disease. Neurology 33:1283–1287PubMed
go back to reference Haller RG, Clausen T, Vissing J (1998) Reduced levels of skeletal muscle Na + K + -ATPase in McArdle disease. Neurology 50:37–40PubMed Haller RG, Clausen T, Vissing J (1998) Reduced levels of skeletal muscle Na + K + -ATPase in McArdle disease. Neurology 50:37–40PubMed
go back to reference Haller RG, Wyrick P, Taivassalo T, Vissing J (2006) Aerobic conditioning: an effective therapy in McArdle’s disease. Ann Neurol 59:922–928CrossRefPubMed Haller RG, Wyrick P, Taivassalo T, Vissing J (2006) Aerobic conditioning: an effective therapy in McArdle’s disease. Ann Neurol 59:922–928CrossRefPubMed
go back to reference Jammes Y, Steinberg JG, Mambrini O, Brégeon F, Delliaux S (2005) Chronic fatigue syndrome: assessment of increased oxidative stress and altered muscle excitability in response to incremental exercise. J Intern Med 257:299–310CrossRefPubMed Jammes Y, Steinberg JG, Mambrini O, Brégeon F, Delliaux S (2005) Chronic fatigue syndrome: assessment of increased oxidative stress and altered muscle excitability in response to incremental exercise. J Intern Med 257:299–310CrossRefPubMed
go back to reference Lewis SF, Haller RG (1986) The pathophysiology of McArdle’s disease: clues to regulation in exercise and fatigue. J Appl Physiol 61:391–401PubMed Lewis SF, Haller RG (1986) The pathophysiology of McArdle’s disease: clues to regulation in exercise and fatigue. J Appl Physiol 61:391–401PubMed
go back to reference Lucia A, Nogales-Gadea G, Perez M, Martin MA, Andreu AL, Arenas J (2008) McArdle disease: what do neurologists need to know? Nat Clin Pract Neurol 4:568–577CrossRefPubMed Lucia A, Nogales-Gadea G, Perez M, Martin MA, Andreu AL, Arenas J (2008) McArdle disease: what do neurologists need to know? Nat Clin Pract Neurol 4:568–577CrossRefPubMed
go back to reference Mate-Munoz JL, Moran M, Perez M, Chamorro-Vina C, Gomez-Gallego F, Santiago C, Chicharro L, Foster C, Nogales-Gadea G, Rubio JC, Andreu AL, Martin MA, Arenas J, Lucia A (2007) Favorable responses to acute and chronic exercise in McArdle patients. Clin J Sport Med 17:297–303CrossRefPubMed Mate-Munoz JL, Moran M, Perez M, Chamorro-Vina C, Gomez-Gallego F, Santiago C, Chicharro L, Foster C, Nogales-Gadea G, Rubio JC, Andreu AL, Martin MA, Arenas J, Lucia A (2007) Favorable responses to acute and chronic exercise in McArdle patients. Clin J Sport Med 17:297–303CrossRefPubMed
go back to reference McArdle B (1951) Myopathy due to a defect in muscle glycogen breakdown. Clin Sci, pp 13–33 McArdle B (1951) Myopathy due to a defect in muscle glycogen breakdown. Clin Sci, pp 13–33
go back to reference Moritani T, Muro M (1987) Motor unit activity and surface electromyogram power spectrum during increasing force of contraction. Eur J Appl Physiol Occup Physiol 56:260–265CrossRefPubMed Moritani T, Muro M (1987) Motor unit activity and surface electromyogram power spectrum during increasing force of contraction. Eur J Appl Physiol Occup Physiol 56:260–265CrossRefPubMed
go back to reference Rubio JC, Garcia-Consuegra I, Nogales-Gadea G, Blazquez A, Cabello A, Lucia A, Andreu AL, Arenas J, Martin MA (2007) A proposed molecular diagnostic flowchart for myophosphorylase deficiency (McArdle disease) in blood samples from Spanish patients. Hum Mutat 28:203–204CrossRefPubMed Rubio JC, Garcia-Consuegra I, Nogales-Gadea G, Blazquez A, Cabello A, Lucia A, Andreu AL, Arenas J, Martin MA (2007) A proposed molecular diagnostic flowchart for myophosphorylase deficiency (McArdle disease) in blood samples from Spanish patients. Hum Mutat 28:203–204CrossRefPubMed
go back to reference Tsujino S, Shanske S, DiMauro S (1993) Molecular genetic heterogeneity of myophosphorylase deficiency (McArdle’s disease). N Engl J Med 329:241–245CrossRefPubMed Tsujino S, Shanske S, DiMauro S (1993) Molecular genetic heterogeneity of myophosphorylase deficiency (McArdle’s disease). N Engl J Med 329:241–245CrossRefPubMed
go back to reference Vissing J, Haller RG (2003a) A diagnostic cycle test for McArdle’s disease. Ann Neurol 54:539–542CrossRefPubMed Vissing J, Haller RG (2003a) A diagnostic cycle test for McArdle’s disease. Ann Neurol 54:539–542CrossRefPubMed
go back to reference Vissing J, Haller RG (2003b) The effect of oral sucrose on exercise tolerance in patients with McArdle’s disease. N Engl J Med 349:2503–2509CrossRefPubMed Vissing J, Haller RG (2003b) The effect of oral sucrose on exercise tolerance in patients with McArdle’s disease. N Engl J Med 349:2503–2509CrossRefPubMed
go back to reference Vissing J, Vissing SF, MacLean DA, Saltin B, Quistorff B, Haller RG (1998) Sympathetic activation in exercise is not dependent on muscle acidosis. Direct evidence from studies in metabolic myopathies. J Clin Invest 101:1654–1660CrossRefPubMed Vissing J, Vissing SF, MacLean DA, Saltin B, Quistorff B, Haller RG (1998) Sympathetic activation in exercise is not dependent on muscle acidosis. Direct evidence from studies in metabolic myopathies. J Clin Invest 101:1654–1660CrossRefPubMed
go back to reference Zange J, Grehl T, Disselhorst-Klug C, Rau G, Muller K, Schroder R, Tegenthoff M, Malin JP, Vorgerd M (2003) Breakdown of adenine nucleotide pool in fatiguing skeletal muscle in McArdle’s disease: a noninvasive 31P-MRS and EMG study. Muscle Nerve 27:728–736CrossRefPubMed Zange J, Grehl T, Disselhorst-Klug C, Rau G, Muller K, Schroder R, Tegenthoff M, Malin JP, Vorgerd M (2003) Breakdown of adenine nucleotide pool in fatiguing skeletal muscle in McArdle’s disease: a noninvasive 31P-MRS and EMG study. Muscle Nerve 27:728–736CrossRefPubMed
Metadata
Title
Excessive skeletal muscle recruitment during strenuous exercise in McArdle patients
Authors
Dale E. Rae
Timothy D. Noakes
Alejandro F. San Juan
Margarita Pérez
Gisela Nogales-Gadea
Jonatan R. Ruiz
María Morán
Miguel A. Martín
Antoni L. Andreu
Joaquín Arenas
Alejandro Lucia
Publication date
01-11-2010
Publisher
Springer-Verlag
Published in
European Journal of Applied Physiology / Issue 5/2010
Print ISSN: 1439-6319
Electronic ISSN: 1439-6327
DOI
https://doi.org/10.1007/s00421-010-1585-5

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