Skip to main content
NCBI home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1989 Sep;52(9):1090–1094. doi: 10.1136/jnnp.52.9.1090

Screening for mitochondrial cytopathies: the sub-anaerobic threshold exercise test (SATET).

L Nashef 1, R J Lane 1
PMCID: PMC1031746  PMID: 2795080

Abstract

A simple test is described for identifying patients with abnormalities of muscle energy metabolism secondary to mitochondrial dysfunction, based on the venous lactate response to exercise at 90% of predicted work rate at the anaerobic threshold. The test was standardised for age, weight and sex of subjects, and was abnormal in all cases of mitochondrial cytopathy tested, with a false positive rate of 7% in a control population. The test was abnormal in two cases of mitochondrial disease in which muscle biopsy was normal or showed only non-specific changes.

Full text

PDF
1090

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Arnold D. L., Taylor D. J., Radda G. K. Investigation of human mitochondrial myopathies by phosphorus magnetic resonance spectroscopy. Ann Neurol. 1985 Aug;18(2):189–196. doi: 10.1002/ana.410180205. [DOI] [PubMed] [Google Scholar]
  2. Aunola S., Rusko H. Aerobic and anaerobic thresholds determined from venous lactate or from ventilation and gas exchange in relation to muscle fiber composition. Int J Sports Med. 1986 Jun;7(3):161–166. doi: 10.1055/s-2008-1025755. [DOI] [PubMed] [Google Scholar]
  3. Bresolin N., Moggio M., Bet L., Gallanti A., Prelle A., Nobile-Orazio E., Adobbati L., Ferrante C., Pellegrini G., Scarlato G. Progressive cytochrome c oxidase deficiency in a case of Kearns-Sayre syndrome: morphological, immunological, and biochemical studies in muscle biopsies and autopsy tissues. Ann Neurol. 1987 Jun;21(6):564–572. doi: 10.1002/ana.410210607. [DOI] [PubMed] [Google Scholar]
  4. Brooke M. H., Carroll J. E., Davis J. E., Hagberg J. M. The prolonged exercise test. Neurology. 1979 May;29(5):636–643. doi: 10.1212/wnl.29.5.636. [DOI] [PubMed] [Google Scholar]
  5. Byrne E., Trounce I. Chronic fatigue and myalgia syndrome: mitochondrial and glycolytic studies in skeletal muscle. J Neurol Neurosurg Psychiatry. 1987 Jun;50(6):743–746. doi: 10.1136/jnnp.50.6.743. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Davis H. A., Cass G. C. The anaerobic threshold as determined before and during lactic acidosis. Eur J Appl Physiol Occup Physiol. 1981;47(2):141–149. doi: 10.1007/BF00421666. [DOI] [PubMed] [Google Scholar]
  7. DiMauro S., Bonilla E., Zeviani M., Nakagawa M., DeVivo D. C. Mitochondrial myopathies. Ann Neurol. 1985 Jun;17(6):521–538. doi: 10.1002/ana.410170602. [DOI] [PubMed] [Google Scholar]
  8. Frackowiak R. S., Herold S., Petty R. K., Morgan-Hughes J. A. The cerebral metabolism of glucose and oxygen measured with positron tomography in patients with mitochondrial diseases. Brain. 1988 Oct;111(Pt 5):1009–1024. doi: 10.1093/brain/111.5.1009. [DOI] [PubMed] [Google Scholar]
  9. Inborn errors of cellular organelles. 24th annual symposium of the SSIEM (Society for the Study of Inborn Errors of Metabolism). Amersfoot, The Netherlands. Proceedings. J Inherit Metab Dis. 1987;10 (Suppl 1):1–200. [PubMed] [Google Scholar]
  10. Johnson M. A., Turnbull D. M., Dick D. J., Sherratt H. S. A partial deficiency of cytochrome c oxidase in chronic progressive external ophthalmoplegia. J Neurol Sci. 1983 Jul;60(1):31–53. doi: 10.1016/0022-510x(83)90125-9. [DOI] [PubMed] [Google Scholar]
  11. Jones N. L., Makrides L., Hitchcock C., Chypchar T., McCartney N. Normal standards for an incremental progressive cycle ergometer test. Am Rev Respir Dis. 1985 May;131(5):700–708. doi: 10.1164/arrd.1985.131.5.700. [DOI] [PubMed] [Google Scholar]
  12. Linderholm H., Müller R., Ringqvist T., Sörnäs R. Hereditary abnormal muscle metabolism with hyperkinetic circulation during exercise. Acta Med Scand. 1969 Mar;185(3):153–166. doi: 10.1111/j.0954-6820.1969.tb07314.x. [DOI] [PubMed] [Google Scholar]
  13. Mastaglia F. L., Thompson P. L., Papadimitriou J. M. Mitochondrial myopathy with cardiomyopathy, lactic acidosis and response to prednisone and thiamine. Aust N Z J Med. 1980 Dec;10(6):660–664. doi: 10.1111/j.1445-5994.1980.tb04250.x. [DOI] [PubMed] [Google Scholar]
  14. Mitsumoto H., Aprille J. R., Wray S. H., Nemni R., Bradley W. G. Chronic progressive external ophthalmoplegia (CPEO): clinical, morphologic, and biochemical studies. Neurology. 1983 Apr;33(4):452–461. doi: 10.1212/wnl.33.4.452. [DOI] [PubMed] [Google Scholar]
  15. Morgan-Hughes J. A., Darveniza P., Kahn S. N., Landon D. N., Sherratt R. M., Land J. M., Clark J. B. A mitochondrial myopathy characterized by a deficiency in reducible cytochrome b. Brain. 1977 Dec;100(4):617–640. doi: 10.1093/brain/100.4.617. [DOI] [PubMed] [Google Scholar]
  16. Ogasahara S., Nishikawa Y., Yorifuji S., Soga F., Nakamura Y., Takahashi M., Hashimoto S., Kono N., Tarui S. Treatment of Kearns-Sayre syndrome with coenzyme Q10. Neurology. 1986 Jan;36(1):45–53. doi: 10.1212/wnl.36.1.45. [DOI] [PubMed] [Google Scholar]
  17. Petty R. K., Harding A. E., Morgan-Hughes J. A. The clinical features of mitochondrial myopathy. Brain. 1986 Oct;109(Pt 5):915–938. doi: 10.1093/brain/109.5.915. [DOI] [PubMed] [Google Scholar]
  18. Poortmans J. R., Delescaille-Vanden Bossche J., Leclercq R. Lactate uptake by inactive forearm during progressive leg exercise. J Appl Physiol Respir Environ Exerc Physiol. 1978 Dec;45(6):835–839. doi: 10.1152/jappl.1978.45.6.835. [DOI] [PubMed] [Google Scholar]
  19. Reinhard U., Müller P. H., Schmülling R. M. Determination of anaerobic threshold by the ventilation equivalent in normal individuals. Respiration. 1979;38(1):36–42. doi: 10.1159/000194056. [DOI] [PubMed] [Google Scholar]
  20. Sherratt H. S., Watmough N. J., Johnson M. A., Turnbull D. M. Methods for study of normal and abnormal skeletal muscle mitochondria. Methods Biochem Anal. 1988;33:243–335. doi: 10.1002/9780470110546.ch6. [DOI] [PubMed] [Google Scholar]
  21. Turnbull D. M., Johnson M. A., Dick D. J., Cartlidge N. E., Sherratt H. S. Partial cytochrome oxidase deficiency without subsarcolemmal accumulation of mitochondria in chronic progressive external ophthalmoplegia. J Neurol Sci. 1985 Aug;70(1):93–100. doi: 10.1016/0022-510x(85)90191-1. [DOI] [PubMed] [Google Scholar]
  22. Ward G. R., MacDougall J. D., Sutton J. R., Toews C. J., Jones N. L. Activation of human muscle pyruvate dehydrogenase with activity and immobilization. Clin Sci (Lond) 1986 Feb;70(2):207–210. doi: 10.1042/cs0700207. [DOI] [PubMed] [Google Scholar]
  23. Wasserman K., Whipp B. J., Davis J. A. Respiratory physiology of exercise: metabolism, gas exchange, and ventilatory control. Int Rev Physiol. 1981;23:149–211. [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES