The Exercise Test

  • N. Kono
  • S. Tarui

Abstract

A “semi-ischemic” forearm exercise test is useful for screening patients with muscle enzyme defects in the metabolic pathway from glycogen (glucose) to lactate. McArdle [1] first described the “ischemic” forearm exercise test; he observed no rise in plasma lactate concentration in the original patient. The test was later modified [2] and has since been used to screen patients with McArdle’s disease (glycogenosis type V). This test, in which the exercising arm is maintained at conditions of complete ischemia, is also available as a diagnostic test for other types of glycogenosis, including types VII [3] and III [4]. However, patients with type V glycogen storage disease (GSD) developed myoglobinemia, massive myoglobinuria, and marked serum creatine kinase elevation subsequent to routinely ischemic forearm exercise tests. Substantial evidence has been accumulated that the ischemic forearm exercise test is potentially hazardous to type V patients, as it might induce massive myoglobinuria sufficient to result in acute myoglobinuric renal failure [5, 6]. Therefore, the test had to be modified in the following fashion [7, 8]: after 30 min rest, blood is drawn from the antecubital vein of the nonexercising arm. A small size sphygmomanometer cuff applied around the wrist of the exercising arm is inflated to 200 mmHg. A second standard cuff around the upper arm is then inflated to mean arterial pressure, and the patient squeezes a hand manometer as powerfully as possible 120 times during a period of 2 min. Immediately after the exercise, the second cuff is rapidly inflated to 200 mmHg. Blood is drawn with a butterfly needle from the antecubital vein of the exercising arm 2 min after the end of the exercise, and the cuff around the upper arm is released. Then blood is obtained every 1 or 2 min four to five times (Table 1). This semi-ischemic forearm exercise test has been used for many patients with types V, VII, and III GSD, including cases of our own, and no severe signs or symptoms appeared during or after the test.

Keywords

Ischemia Lactate Creatine Glucagon Purine 

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References

  1. 1.
    McArdle B (1951) Myopathy due to a defect in muscle glycogen breakdown. Clin Sci 10: 13–33Google Scholar
  2. 2.
    Schmid R, Mahler R (1959) Chronic progressive myopathy with myoglobinuria: demonstration of glycogenolytic defect in muscle. J Clin Invest 38: 2044–2058PubMedCrossRefGoogle Scholar
  3. 3.
    Tarui S, Okuno G, Ikura Y, Tanaka T, Suda M, Nishikawa M (1965) Phosphofructokinase deficiency in skeletal muscle: a new type of glycogenosis. Biochem Biophys Res Commun 19: 517–523PubMedCrossRefGoogle Scholar
  4. 4.
    Ozand P, Tokatli M, Amiri S (1967) Biochemical investigation of an unusual case of glycogenosis. J Pediatr 71: 225–232PubMedCrossRefGoogle Scholar
  5. 5.
    Neumann P (1982) Scintigraphic demonstration of a iatrogenic rhabdomyolysis in McArdle’s syndrome. Nukl Med 21: 12–15Google Scholar
  6. 6.
    Meinck HM, Goebel HH, Rumpf KW, Kaiser H, Neumann P (1982) The forearm ischaemic work test–hazardous to McArdle patients? J Neurol Neurosurg Psychiatry 45: 1144–1146PubMedCrossRefGoogle Scholar
  7. 7.
    Kono N, Mineo I, Sumi S, Shimizu T, Kang J, Nonaka K, Tarui S (1984) Metabolic basis of improved exercise tolerance: muscle phosphorylase deficiency after glucagon administration. Neurology 34: 1471–1476PubMedCrossRefGoogle Scholar
  8. 8.
    Mineo I, Kono N, Shimizu T, Sumi S, Nonaka K, Tarui S (1984) A comparative study on glucagon effect between McArdle disease and Tarui disease. Muscle Nerve 7: 552–559PubMedCrossRefGoogle Scholar
  9. 9.
    Rumpf KW, Wagner H, Kaiser H, Meinck HM, Goebel HH, Scheler F (1981) Increased ammonia production during forearm ischemic work test in McArdle’s disease. Klin Wochenschr 59: 1319–1320PubMedCrossRefGoogle Scholar
  10. 10.
    Mineo I, Kono N, Shimizu T, Hara N, Yamada Y, Sumi S, Nonaka K, Tarui S (1985) Excess purine degradation in exercising muscles of patients with glycogen storage disease types V and VII. J Clin Invest 76: 556–559PubMedCrossRefGoogle Scholar
  11. 11.
    Brooke MH, Patterson VH, Kaiser KK (1983) Hypoxanthine and McArdle disease: a clue to metabolic stress in the working forearm. Muscle Nerve 6: 204–206PubMedCrossRefGoogle Scholar
  12. 12.
    Kono N, Mineo I, Shimizu T, Hara N, Yamada Y, Nonaka K, Tarui S (1986) Increased plasma uric acid after exercise in muscle phosphofructokinase deficiency. Neurology 36: 106–108PubMedCrossRefGoogle Scholar
  13. 13.
    Mineo I, Kono N, Hara N, Shimizu T, Yamada Y, Kawachi M, Kiyokawa H, Wang YL, Tarui S (1987) Myogenic hyperuricemia. A common pathophysiologic feature of glycogenosis types III, V and VH. N Engl J Med 317: 75–80PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1990

Authors and Affiliations

  • N. Kono
  • S. Tarui

There are no affiliations available

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