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Pulmonary function and resting breathing pattern in myotonic dystrophy

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Abstract

In 17 patients with myotonic dystrophy, spirometric, flow-volume, and maximal mouth occlusion variables were obtained and compared with 8 normal subjects. Ventilatory CO2, response was measured by the estimation of the steady-state effect of a sufficiently large serial dead space. Variability of resting breathing pattern was expressed by the variation coefficients of respiratory cycle time and tidal volume.

The group means of the total lung capacity (TLC), vital capacity (VC), forced expiratory volume in 1 sec (FEV)1, and forced inspiratory volume in 1 sec (FIV)1, showed a restrictive pattern. Only maximal static mouth pressure (Pi,max), measured at residual volume (RV) level, showed a significant positive correlation with both VC (p = 0.03) and FIV1, (p = 0.02), suggesting inspiratory muscle weakness as a determinant of the restriction.

Although the differences were just not significant, both variation coefficients of the respiratory cycle time and tidal volume were larger in the group with a CO, sensitivity below the lower limit of normal compared to those with a normal ventilatory response to CO2. In 3 patients, fluctuations in FRC were also present. We hypothesize that, in addition to the already documented FRC fluctuations by uncoordinated spontaneous intercostal muscle action, a defect of integration of afferent neural input and chemical drive in the medullary region may also be present in these patients.

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References

  1. Askanazi J, Silverberg P, Hijman Al, Foster RJ, Yaremchuk M, Kinney JM (1978) Effects of the mask and mouthpiece plus noseclip on spontaneous breating pattern. Crit Care Med, 6(3):143–146

    Google Scholar 

  2. Askanazi J, Silverberg PA, Foster RJ, Hijman Al, Milic-Emili J, Kinney JM (1980) Effects of respiratory apparatus on breathing pattern. J Appl Physiol Respir Environ Exercise Physiol 48(4):577–580

    Google Scholar 

  3. Begin R, Bureau MA, Lupien L, Lemieux B (1980) Control and modulation of respiration in Steinert's myotonic dystrophy. Am Rev Respir Dis 121:281–289

    Google Scholar 

  4. Begin R, Bureau MA, Lupien L, Bernier JP, Lemieux B (1982) Pathogenesis of respiratory insufficiency in myotonic dystrophy. The mechanical factors. Am Rev Respir Dis 125:312–318

    Google Scholar 

  5. Berkenbosch A, Bovill JG, Dahan A, Goede J de, Olivier ICW (1989) The ventilatory CO2 sensitivities from Read's rebreathing method and the steady-state method are not equal in man. J Physiol 411:367–377

    Google Scholar 

  6. Bird TD, Follett C, Griep E (1983) Cognitive and personality function in myotonic muscular dystrophy. J Neurol Neurosurg Psychiatry 46:971–980

    Google Scholar 

  7. Black WC, Ravin A (1947) Studies in dystrophia myotonica. Autopsy observations in 5 cases. Arch Pathol 44:176–191

    Google Scholar 

  8. Burki NK (1989) Measurements of ventilatory regulation. Clin Chest Med 10(2):215–226

    Google Scholar 

  9. Caroll JE, Zwillich CW, Weil JV (1977) Ventilatory response in myotonic dystrophy. Neurology 27:1125–1128

    Google Scholar 

  10. Gilbert R, Howland Auchincloss J, Brodsky J, Boden J (1972) Changes in tidal volume, frequency and ventilation induced by their measurement. J Appl Physiol 33(2):252–254

    Google Scholar 

  11. Gillam PMS, Heaf PJD, Kaufman L, Lucas BGB (1964) Respiration in dystrophia myotonica. Thorax 19:112–120

    Google Scholar 

  12. Gribbin HR, Gardiner IT, Heinz GD, Gibson GJ, Pride NM (1983) Role of impaired inspiratory muscle function in limiting the ventilatory response to carbon dioxide in chronic airflow obstruction. Clin Sci 64:487–495

    Google Scholar 

  13. Hirsch JA, Bishop B (1982) Human breathing patterns on mouthpiece or face mask during air, CO2 or low O2. J Appl Physiol Respir Environ Exercise Physiol 53(5):1281–1290

    Google Scholar 

  14. Jammes Y, Pouget J, Grimaud C, Serratrice G (1985) Pulmonary function and electromyographic study of respiratory muscles in myotonic dystrophy. Muscle Nerve 8:586–594

    Google Scholar 

  15. Kilburn KH, Eagan JT, Sieker HO, Heijman A (1959) Cardiopulmonary insufficiency in myotonic and progressive muscular dystrophy. N Engl J Med 261(22):1089–1096

    Google Scholar 

  16. Meché FGA van der, Bogaard JM, Berg B van den (1986) Treatment of hypersomnolence in myotonic dystrophy with a CNS stimulant. Muscle Nerve 9:341–344

    Google Scholar 

  17. Quanjer PH (1983) Standardized lung function testing. Bull Eur Physiopathol Respir 19 (suppl 5)

  18. Rochester DF, Arora NS (1983) Respiratory muscle failure. Med Clin North Am 67:573–597

    Google Scholar 

  19. Sackner JD, Nixon AJ, Davis B, Atkins N, Sackner MA (1980) Effects of breathing through external dead space on ventilation at rest and during exercise II. Am Rev Respir Dis 122:933–940

    Google Scholar 

  20. Serisier DE, Mastaglia FL, Gibson GJ (1982) Respiratory muscle function and ventilatory control: I, In patients with motor neurone disease; II, in patients with myotonic dystrophy. Q J Med 51:205–226

    Google Scholar 

  21. Snedecor GW, Cochran WG (1967) Statistical methods, 6th ed. Ames, IA: Iowa State University Press

    Google Scholar 

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Bogaard, J.M., van der Meche, F.G.A., Hendriks, I. et al. Pulmonary function and resting breathing pattern in myotonic dystrophy. Lung 170, 143–153 (1992). https://doi.org/10.1007/BF00174317

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  • DOI: https://doi.org/10.1007/BF00174317

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