Abstract
Background: Sleep disordered breathing (SDB) is common in neuromuscular diseases but its relationship to respiratory function is poorly defined. A study was undertaken to identify distinct patterns of SDB, to clarify the relationships between SDB and lung and respiratory muscle function, and to identify daytime predictors for SDB at its onset, for SDB with continuous hypercapnic hypoventilation, and for diurnal respiratory failure.
Methods: Upright and supine inspiratory vital capacity (IVC, % predicted), maximal inspiratory muscle pressure (PImax), respiratory drive (P0.1), respiratory muscle effort (P0.1/PImax), and arterial blood gas tensions were prospectively compared with polysomnography and capnometry (PtcCO2) in 42 patients with primary myopathies.
Results: IVC correlated with respiratory muscle function and gas exchange by day and night. SDB evolved in three distinct patterns from REM hypopnoeas, to REM hypopnoeas with REM hypoventilation, to REM/non-REM (continuous) hypoventilation, and preceded diurnal respiratory failure. SDB correlated with IVC and PImax which yielded highly predictive thresholds for SDB onset (IVC <60%, PImax <4.5 kPa), SDB with continuous hypoventilation (IVC <40%, PImax <4.0 kPa), and SDB with diurnal respiratory failure (IVC <25%, PImax <3.5 kPa).
Conclusion: Progressive ventilatory restriction in neuromuscular diseases correlates with respiratory muscle weakness and results in progressive SDB which, by pattern and severity, can be predicted from daytime lung and respiratory muscle function.
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Selected References
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- Barbé F., Quera-Salva M. A., McCann C., Gajdos P., Raphael J. C., de Lattre J., Agustí A. G. Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy. Eur Respir J. 1994 Aug;7(8):1403–1408. doi: 10.1183/09031936.94.07081403. [DOI] [PubMed] [Google Scholar]
- Braun N. M., Arora N. S., Rochester D. F. Respiratory muscle and pulmonary function in polymyositis and other proximal myopathies. Thorax. 1983 Aug;38(8):616–623. doi: 10.1136/thx.38.8.616. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Broughton R., Stuss D., Kates M., Roberts J., Dunham W. Neuropsychological deficits and sleep in myotonic dystrophy. Can J Neurol Sci. 1990 Nov;17(4):410–415. doi: 10.1017/s0317167100030985. [DOI] [PubMed] [Google Scholar]
- Bye P. T., Ellis E. R., Issa F. G., Donnelly P. M., Sullivan C. E. Respiratory failure and sleep in neuromuscular disease. Thorax. 1990 Apr;45(4):241–247. doi: 10.1136/thx.45.4.241. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Criée C. P. Analysis of inspiratory mouth pressures. Prax Klin Pneumol. 1988 Dec;42 (Suppl 2):820–826. [PubMed] [Google Scholar]
- Finnimore A. J., Jackson R. V., Morton A., Lynch E. Sleep hypoxia in myotonic dystrophy and its correlation with awake respiratory function. Thorax. 1994 Jan;49(1):66–70. doi: 10.1136/thx.49.1.66. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Hahn A., Bach J. R., Delaubier A., Renardel-Irani A., Guillou C., Rideau Y. Clinical implications of maximal respiratory pressure determinations for individuals with Duchenne muscular dystrophy. Arch Phys Med Rehabil. 1997 Jan;78(1):1–6. doi: 10.1016/s0003-9993(97)90001-0. [DOI] [PubMed] [Google Scholar]
- Howard R. S., Wiles C. M., Hirsch N. P., Spencer G. T. Respiratory involvement in primary muscle disorders: assessment and management. Q J Med. 1993 Mar;86(3):175–189. [PubMed] [Google Scholar]
- Hukins C. A., Hillman D. R. Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy. Am J Respir Crit Care Med. 2000 Jan;161(1):166–170. doi: 10.1164/ajrccm.161.1.9901057. [DOI] [PubMed] [Google Scholar]
- Khan Y., Heckmatt J. Z. Obstructive apnoeas in Duchenne muscular dystrophy. Thorax. 1994 Feb;49(2):157–161. doi: 10.1136/thx.49.2.157. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Labanowski M., Schmidt-Nowara W., Guilleminault C. Sleep and neuromuscular disease: frequency of sleep-disordered breathing in a neuromuscular disease clinic population. Neurology. 1996 Nov;47(5):1173–1180. doi: 10.1212/wnl.47.5.1173. [DOI] [PubMed] [Google Scholar]
- Marcus C. L., Omlin K. J., Basinki D. J., Bailey S. L., Rachal A. B., Von Pechmann W. S., Keens T. G., Ward S. L. Normal polysomnographic values for children and adolescents. Am Rev Respir Dis. 1992 Nov;146(5 Pt 1):1235–1239. doi: 10.1164/ajrccm/146.5_Pt_1.1235. [DOI] [PubMed] [Google Scholar]
- Mellies U., Ragette R., Schwake C., Baethmann M., Voit T., Teschler H. Sleep-disordered breathing and respiratory failure in acid maltase deficiency. Neurology. 2001 Oct 9;57(7):1290–1295. doi: 10.1212/wnl.57.7.1290. [DOI] [PubMed] [Google Scholar]
- Mier-Jedrzejowicz A., Brophy C., Moxham J., Green M. Assessment of diaphragm weakness. Am Rev Respir Dis. 1988 Apr;137(4):877–883. doi: 10.1164/ajrccm/137.4.877. [DOI] [PubMed] [Google Scholar]
- Phillips M. F., Smith P. E., Carroll N., Edwards R. H., Calverley P. M. Nocturnal oxygenation and prognosis in Duchenne muscular dystrophy. Am J Respir Crit Care Med. 1999 Jul;160(1):198–202. doi: 10.1164/ajrccm.160.1.9805055. [DOI] [PubMed] [Google Scholar]
- Quanjer P. H., Tammeling G. J., Cotes J. E., Pedersen O. F., Peslin R., Yernault J. C. Lung volumes and forced ventilatory flows. Report Working Party Standardization of Lung Function Tests, European Community for Steel and Coal. Official Statement of the European Respiratory Society. Eur Respir J Suppl. 1993 Mar;16:5–40. [PubMed] [Google Scholar]
- Quera-Salva M. A., Guilleminault C., Chevret S., Troche G., Fromageot C., Crowe McCann C., Stoos R., de Lattre J., Raphael J. C., Gajdos P. Breathing disorders during sleep in myasthenia gravis. Ann Neurol. 1992 Jan;31(1):86–92. doi: 10.1002/ana.410310116. [DOI] [PubMed] [Google Scholar]
- Simonds A. K., Muntoni F., Heather S., Fielding S. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax. 1998 Nov;53(11):949–952. doi: 10.1136/thx.53.11.949. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Van Lunteren Erik, Kaminski Henry J. Disorders of Sleep and Breathing during Sleep in Neuromuscular Disease. Sleep Breath. 1999;3(1):23–30. doi: 10.1007/s11325-999-0023-6. [DOI] [PubMed] [Google Scholar]
- White J. E., Drinnan M. J., Smithson A. J., Griffiths C. J., Gibson G. J. Respiratory muscle activity and oxygenation during sleep in patients with muscle weakness. Eur Respir J. 1995 May;8(5):807–814. [PubMed] [Google Scholar]