A diagnostic cycle test for McArdle's disease

We investigated whether the second wind phenomenon (ie, a decrease in heart rate and perceived exertion during exercise) is pathognomonic for McArdle's disease. Twenty‐four patients with McArdle's disease, 17 healthy subjects, and 25 patients with other inborn errors of muscle metabolism c...

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Published in	Annals of neurology Vol. 54; no. 4; pp. 539 - 542
Main Authors	Vissing, John, Haller, Ronald G.
Format	Journal Article
Language	English
Published	Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.10.2003 Willey-Liss
Subjects	Biological and medical sciences Carbohydrates (enzymatic deficiencies). Glycogenosis Case-Control Studies Errors of metabolism Exercise - physiology Exercise Test Exercise Therapy Female Glycogen Storage Disease Type V - diagnosis Glycogen Storage Disease Type V - physiopathology Heart Rate - physiology Humans Male Medical sciences Metabolic diseases Metabolism, Inborn Errors - diagnosis Metabolism, Inborn Errors - physiopathology Predictive Value of Tests Reproducibility of Results Time Factors Physical exercise Human Glycogenosis V Mac Ardle Healthy subject Cardiovascular disease Metabolic diseases Metabolism Enzymopathy Genetic disease Workload Heart rate Heart disease Carbohydrate Diagnosis
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ISSN	0364-5134 1531-8249
DOI	10.1002/ana.10725

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Abstract	We investigated whether the second wind phenomenon (ie, a decrease in heart rate and perceived exertion during exercise) is pathognomonic for McArdle's disease. Twenty‐four patients with McArdle's disease, 17 healthy subjects, and 25 patients with other inborn errors of muscle metabolism cycled a constant workload for 15 minutes. In McArdle's disease patients, heart rate consistently decreased by 35 ± 3 beats per minute from the 7th to the 15th minute of exercise, whereas heart rate increased progressively with exercise in all 42 control subjects. The findings indicate that cycling at a moderate, constant workload provides a specific, sensitive, and simple diagnostic test for McArdle's disease. Ann Neurol 2003;54:539‐542
AbstractList	We investigated whether the second wind phenomenon (ie, a decrease in heart rate and perceived exertion during exercise) is pathognomonic for McArdle's disease. Twenty‐four patients with McArdle's disease, 17 healthy subjects, and 25 patients with other inborn errors of muscle metabolism cycled a constant workload for 15 minutes. In McArdle's disease patients, heart rate consistently decreased by 35 ± 3 beats per minute from the 7th to the 15th minute of exercise, whereas heart rate increased progressively with exercise in all 42 control subjects. The findings indicate that cycling at a moderate, constant workload provides a specific, sensitive, and simple diagnostic test for McArdle's disease. Ann Neurol 2003;54:539‐542 We investigated whether the second wind phenomenon (ie, a decrease in heart rate and perceived exertion during exercise) is pathognomonic for McArdle's disease. Twenty‐four patients with McArdle's disease, 17 healthy subjects, and 25 patients with other inborn errors of muscle metabolism cycled a constant workload for 15 minutes. In McArdle's disease patients, heart rate consistently decreased by 35 ± 3 beats per minute from the 7 th to the 15 th minute of exercise, whereas heart rate increased progressively with exercise in all 42 control subjects. The findings indicate that cycling at a moderate, constant workload provides a specific, sensitive, and simple diagnostic test for McArdle's disease. Ann Neurol 2003;54:539‐542 We investigated whether the second wind phenomenon (ie, a decrease in heart rate and perceived exertion during exercise) is pathognomonic for McArdle's disease. Twenty-four patients with McArdle's disease, 17 healthy subjects, and 25 patients with other inborn errors of muscle metabolism cycled a constant workload for 15 minutes. In McArdle's disease patients, heart rate consistently decreased by 35 +/- 3 beats per minute from the 7(th) to the 15(th) minute of exercise, whereas heart rate increased progressively with exercise in all 42 control subjects. The findings indicate that cycling at a moderate, constant workload provides a specific, sensitive, and simple diagnostic test for McArdle's disease.We investigated whether the second wind phenomenon (ie, a decrease in heart rate and perceived exertion during exercise) is pathognomonic for McArdle's disease. Twenty-four patients with McArdle's disease, 17 healthy subjects, and 25 patients with other inborn errors of muscle metabolism cycled a constant workload for 15 minutes. In McArdle's disease patients, heart rate consistently decreased by 35 +/- 3 beats per minute from the 7(th) to the 15(th) minute of exercise, whereas heart rate increased progressively with exercise in all 42 control subjects. The findings indicate that cycling at a moderate, constant workload provides a specific, sensitive, and simple diagnostic test for McArdle's disease. We investigated whether the second wind phenomenon (ie, a decrease in heart rate and perceived exertion during exercise) is pathognomonic for McArdle's disease. Twenty-four patients with McArdle's disease, 17 healthy subjects, and 25 patients with other inborn errors of muscle metabolism cycled a constant workload for 15 minutes. In McArdle's disease patients, heart rate consistently decreased by 35 +/- 3 beats per minute from the 7(th) to the 15(th) minute of exercise, whereas heart rate increased progressively with exercise in all 42 control subjects. The findings indicate that cycling at a moderate, constant workload provides a specific, sensitive, and simple diagnostic test for McArdle's disease.
Author	Vissing, John Haller, Ronald G.
Author_xml	– sequence: 1 givenname: John surname: Vissing fullname: Vissing, John email: vissing@rh.dk organization: Department of Neurology and Copenhagen Muscle Research Center, National University Hospital, Rigshospitalet, Copenhagen, Denmark – sequence: 2 givenname: Ronald G. surname: Haller fullname: Haller, Ronald G. organization: Neuromuscular Center, Institute for Exercise and Environmental Medicine, Presbyterian Hospital and Department of Neurology, VA Medical Center and UT Southwestern Medical Center, Dallas, TX
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Keywords	Physical exercise Human Glycogenosis V Mac Ardle Healthy subject Cardiovascular disease Metabolic diseases Metabolism Enzymopathy Genetic disease Workload Heart rate Heart disease Carbohydrate Diagnosis
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References	Vissing J, Galbo H, Haller RG. Paradoxically enhanced glucose production during exercise in humans with blocked glycolysis due to muscle phosphofructokinase deficiency. Neurology 1996; 47: 766-771. Felig P, Wahren J. Fuel homeostasis in exercise. N Engl J Med 1975; 293: 1078-1084. Meinck HM, Goebel HH, Rumpf KW, et al. The forearm ischaemic work test-hazardous to McArdle patients? J Neurol Neurosurg Psych 1982; 45: 1144-1146. Nielsen JN, Wojtaszewski JFP, Haller RG, et al. Role of 5′AMP-activated protein kinase in glycogen synthase activity and glucose utilization: insights from patients with McArdle's disease. J Physiol (Lond) 2002; 541: 979-989. Ørngreen MC, Olsen DB, Vissing J. Exercise tolerance in carnitine palmitoyltransferase II deficiency with intravenous and oral glucose. Neurology 2002; 59: 1046-1051. Åstrand P, Rodahl K. Textbook of work physiology: physiological bases of exercise: New York: McGraw-Hill, 1986. Vissing J, Lewis SF, Galbo H, Haller RG. Effect of deficient muscular glycogenolysis on extramuscular fuel production in exercise. J Appl Physiol 1992; 72: 1773-1779. Vissing J, Gansted U, Quistorff B. Exercise intolerance in mitochondrial myopathy is not related to lactic acidosis. Ann Neurol 2001; 49: 672-676. Kazemi-Esfarjani P, Skomorowska E, Jensen TD, et al. A nonischemic forearm exercise test for McArdle disease. Ann Neurol 2002; 52: 153-159. Lindner A, Reichert N, Eichhorn M, Zierz S. Acute compartment syndrome after forearm ischemic work test in a patient with McArdle's disease. Neurology 2001; 56: 1779-1780. Åstrand I. Aerobic work capacity in men and women with special reference to age. Acta Physiol Scand 1960; 49(suppl 169 1-67). Braakhekke JP, deBruin MI, Stegeman DF, et al. The second wind phenomenon in McArdle's disease. Brain 1986; 109: 1087-1101. Pearson C, Rimer D, Mommaerts WFHM. A metabolic myopathy due to absence of muscle phosphorylase. Am J Med 1961; 30: 502-517. Haller RG, Vissing J. Absence of a spontaneous second wind in muscle phosphofructokinase deficiency. Neurology 2001; 56(suppl 3): A231. Jensen TD, Kazemi-Esfarjani P, Skomorowska E, Vissing J. A forearm exercise screening test for mitochondrial myopathy. Neurology 2002; 58: 1533-1538. Borg, G. Perceived exertion as an indicator of somatic stress. Scand J Rehab Med 1970; 2: 92-98. Nielsen JN, Vissing J, Wojtaszewski JFP, et al. Decreased insulin action in skeletal muscle from patients with McArdle's disease. Am J Physiol 2002; 282: E1267-E1275. Haller RG, Vissing J. Spontaneous "second wind" and glucose-induced second "second wind" in McArdle disease-oxidative mechanisms. Arch Neurol 2002; 59: 1395-1402. 1982; 45 1986; 109 2002; 58 2002; 59 2002; 282 2001 2002; 52 1960; 49 2002; 541 1986 1975; 293 1994 1961; 30 2001; 49 1996; 47 2001; 56 1992; 72 1970; 2 Vissing J (e_1_2_6_4_2) 2001 e_1_2_6_20_2 Haller RG (e_1_2_6_19_2) 2001; 56 Åstrand P (e_1_2_6_14_2) 1986 e_1_2_6_8_2 Åstrand I (e_1_2_6_15_2) 1960; 49 e_1_2_6_7_2 e_1_2_6_18_2 Vissing J (e_1_2_6_16_2) 1992; 72 e_1_2_6_6_2 e_1_2_6_5_2 e_1_2_6_12_2 DiMauro S (e_1_2_6_3_2) 1994 e_1_2_6_13_2 e_1_2_6_2_2 e_1_2_6_10_2 (e_1_2_6_9_2) 1970; 2 e_1_2_6_11_2 e_1_2_6_21_2 e_1_2_6_17_2
References_xml	– reference: Nielsen JN, Wojtaszewski JFP, Haller RG, et al. Role of 5′AMP-activated protein kinase in glycogen synthase activity and glucose utilization: insights from patients with McArdle's disease. J Physiol (Lond) 2002; 541: 979-989. – reference: Jensen TD, Kazemi-Esfarjani P, Skomorowska E, Vissing J. A forearm exercise screening test for mitochondrial myopathy. Neurology 2002; 58: 1533-1538. – reference: Åstrand I. Aerobic work capacity in men and women with special reference to age. Acta Physiol Scand 1960; 49(suppl 169 1-67). – reference: Nielsen JN, Vissing J, Wojtaszewski JFP, et al. Decreased insulin action in skeletal muscle from patients with McArdle's disease. Am J Physiol 2002; 282: E1267-E1275. – reference: Ørngreen MC, Olsen DB, Vissing J. Exercise tolerance in carnitine palmitoyltransferase II deficiency with intravenous and oral glucose. Neurology 2002; 59: 1046-1051. – reference: Braakhekke JP, deBruin MI, Stegeman DF, et al. 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Snippet	We investigated whether the second wind phenomenon (ie, a decrease in heart rate and perceived exertion during exercise) is pathognomonic for McArdle's...
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SubjectTerms	Biological and medical sciences Carbohydrates (enzymatic deficiencies). Glycogenosis Case-Control Studies Errors of metabolism Exercise - physiology Exercise Test Exercise Therapy Female Glycogen Storage Disease Type V - diagnosis Glycogen Storage Disease Type V - physiopathology Heart Rate - physiology Humans Male Medical sciences Metabolic diseases Metabolism, Inborn Errors - diagnosis Metabolism, Inborn Errors - physiopathology Predictive Value of Tests Reproducibility of Results Time Factors
Title	A diagnostic cycle test for McArdle's disease
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