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Lactate Stress Testing in 155 Patients with Mitochondriopathy

Published online by Cambridge University Press:  02 December 2014

Josef Finsterer
Affiliation:
Ludwig Boltzmann Institute for Research in Neuromuscular Disorders, Vienna, Austria, Europe
Erika Milvay
Affiliation:
Ludwig Boltzmann Institute for Research in Neuromuscular Disorders, Vienna, Austria, Europe
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Abstract

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Objective:

Few data are available about the diagnostic yield of the lactate stress test (LST) in a large group of patients with mitochondriopathy (MCP).

Methods:

Serum lactate was determined once before, three times during, and once after a 15-minute, constant 30W workload on a bicycle in 62 controls, aged 17 to 84 years, 155 patients with MCP, aged 17 to 87 years, and 31 patients with neurological disorders other than MCP.

Results:

Lactate's upper reference limits at rest, 5, 10, 15 minutes after starting, and 15 minutes after finishing the exercise were 2.0, 2.1, 2.1, 2.1 and 1.8 mmol/l respectively. The test was regarded abnormal if more than two of the five lactate values exceeded the cut-off levels. Among the 103 patients with abnormal LST, 64 (62 %) had normal resting lactate. The sensitivity of the test was 67% and the specificity 94%.

Conclusion:

The LST proved to have a high sensitivity and specificity in the detection of patients with MCP, being thus a simple but powerful tool to assess the impaired oxidative metabolism in MCP patients.

Résumé:

RÉSUMÉ:Objectif:

Il y a peu de données disponibles sur la valeur diagnostique du test du lactate à l'effort (TLE) chez un groupe important de patients atteints de mitochondriopathie (MCP).

Méthodes:

Le taux de lactate sérique a été déterminé avant, à trois reprises pendant et après un e activité physique de 15 minutes, d'intensité constante à 30W sur une bicyclette, chez 62 contrôles âgés de 17 à 84 ans, 155 patients atteints de MCP, âgés de 17 à 87 ans et 31 patients présentant d'autres pathologies neurologiques.

Résultats:

Le point de coupe supérieur des valeurs de référence au repos, 5, 10, 15 minutes après le début de l'exercice et 15 minutes après la fin de l'exercice étaient de 2,0, 2,1, 2,1, 2,1 et 1,8 mmol/L respectivement. Le test était considéré comme normal si plus de deux des cinq valeurs de lactate étaient supérieures au point de coupe. Parmi les 103 patients dont le TLE était anormal, 64 (62%) avaient un taux de lactate normal au repos. La sensibilité du test était de 67% et la spécificité de 94%.

Conclusion:

Le TLE a démontré une sensibilité et une spécificité élevées pour la détection de patients porteurs d'une MCP. C'est un outil simple mais puissant pour évaluer l'altération du métabolisme oxydatif chez les patients atteints de MCP.

Type
Original Article
Copyright
Copyright © Canadian Neurological Sciences Federation 2002

References

1. DiMauro, S, Bonilla, E, Zeviani, M, Nakagawa, M, DeVivo, DC. Mitochondrial myopathies. Ann Neurol 1985: 17: 521538.CrossRefGoogle ScholarPubMed
2. Morgan-Hughes, JA. The mitochondrial myopathies. In: Engel, AG, Franzini-Armstrong C, eds. Myology. Basic and Clinical. New York: McGraw-Hill, 1994: 16101651.Google Scholar
3. Finsterer, J, Shorny, S, Capek, J, et al. Lactate-stress test in thediagnosis of mitochondrial myopathy. J Neurol Sci 1998: 159:176180.CrossRefGoogle Scholar
4. Finsterer, J, Eichberger, H, Jarius, C. Lactate stress testing in 54 patients with mitochondriopathy. Eur Arch Psych Clin Neuro sci 2000: 250: 3639.CrossRefGoogle ScholarPubMed
5. Hashef, L, Lane, RJ. Screening for mitochondrial cytopathies: thesub-anaerobic threshold exercise test (SATET). J NeurolNeurosurg Psychiatry 1989: 52: 10901094.Google Scholar
6. Land, JM, Hockaday, JM, Hughes, JT, Ross, BD. Childhoodmitochondrial myopathy with ophthalmoplegia. J Neurol Sci 1981: 51: 371382.CrossRefGoogle ScholarPubMed
7. Shapira, AHV, DiMauro, S. Mitochondrial Disorders in Neurology. Oxford: Butterworth Heinemann, 1994: 7576.CrossRefGoogle Scholar
8. Zierz, S, Meeßen, S, Jerusalem, F. Laktat- und Pyruvatspiegel in derDiagnostik mitochondrialer Myopathien. Nervenarzt 1989: 60: 545548.Google Scholar
9. Haller, RG, Bertocci, LA. Exercise evaluation of metabolicmyopathies. In: Engel, AG, Franzini-Armstrong C, eds. Myology. Basic and Clinical. New York: McGraw-Hill, 1994: 807821.Google Scholar
10. Chan, A, Gold, R, Arp, S, et al. A standardised bicycle ergometer testin the diagnosis and monitoring of mitochondrial myopathies. Nervenarzt 1998: 69: 472484.CrossRefGoogle Scholar
11. Dengler, R, Wohlfarth, K, Zierz, S, Jöbges, M, Schubert, M. Musclefatigue, lactate, and pyruvate in mitochondrial myopathy with progressive external ophthalmoplegia. Muscle Nerve 1996: 19: 456462.3.0.CO;2-B>CrossRefGoogle Scholar
12. Petty, RKH, Harding, AE, Morgan-Hughes, JA. The clinical featuresof mitochondrial myopathy. Brain 1986: 109: 915938.CrossRefGoogle Scholar
13. Vissing, J, Galbo, H, Haller, RG. Exercise fuel mobilisation inmitochondrial myopathy: a metabolic dilemma. Ann Neurol 1996: 40: 655662.CrossRefGoogle Scholar
14. Shapira, Y, Cederbaum, SD, Cancilla, PA, Nielsen, D, Lippe, BM. Familial poliodystrophy, mitochondrial myopathy, and lactate acidaemia. Neurology 1975: 25: 614621.CrossRefGoogle Scholar
15. Collins, S, Byrne, E, Dennett, X. Contrasting histochemical featuresof various mitochondrial syndromes. Acta Neurol Scand 1995; 91: 287293.CrossRefGoogle Scholar
16. Schmidt, M, Kunkel, M, Schuff-Werner, P, et al. Standardised aerobictreadmill ergometry in healthy subjects and patients with mitochondrial and non-mitochondrial myopathies. Nervenarzt 1997; 68: 831835.CrossRefGoogle Scholar
17. Johnson, MA, Turnbull, DM, Dick, DJ, Sherratt, HSA. A partialdeficiency of cytochrome C oxidase in chronic progressive external ophthalmoplegia. J Neurol Sci 1983; 60: 3135.CrossRefGoogle Scholar
18. Ogasahara, S, Nishikawa, Y, Yorifuji, S, et al. Treatment of Kearns-Sayre syndrome with coenzyme Q10. Neurology 1986; 36: 4553.CrossRefGoogle ScholarPubMed
19. Sengers, RCA, ter Haar, BGA, Trijbels, JMF, et al. Congenitalcataract and mitochondrial myopathy of skeletal and heart muscle associated with lactic acidosis after exercise. J Pediatrics 1975; 86: 873880.CrossRefGoogle Scholar
20. Siciliano, G, Renna, M, Nanca, ML, et al. The relationship of plasmacatecholamine and lactate during anaerobic threshold exercise in mitochondrial myopathies. Neuromusc Disord 1999; 9: 411416.CrossRefGoogle Scholar
21. Jackson, MJ, Schaefer, JA, Johnson, MA, et al. Presentation andclinical investigation of mitochondrial respiratory chain disease. A study of 51 patients. Brain 1995; 118: 339357.Google Scholar
22. Finsterer, J, Milvay, E. Diagnostic yield of the lactate stress testunder absolute or relative workload in respiratory chaindisorders. J Neurosci Meth 2001;108:6570.CrossRefGoogle Scholar