Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy

Sleep-related respiratory disturbances (SRD) in patients with muscle diseases may have significant clinical implications, because the patients frequently die at night. The aims of the study were to :1) assess the presence and severity of sleep-related respiratory disturbances in patients with Duchenne muscular distrophy (DMD); and 2) investigate the relationship of sleep-related respiratory disturbances to daytime symptoms and pulmonary function. We studied six clinically stable patients with Duchenne muscular dystrophy, mean age (+/- SD) 18 +/- 2 yrs. Vital capacity was 27 +/- 19% of predicted and daytime arterial oxygen tension (PaO2) was 10.9 +/- 1 kPa (range 8.9-12.4 kPa). The presence of daytime somnolence, insomnia, headache, nightmares and/or snoring was recorded. Four patients (67%) showed symptoms that suggest sleep-related respiratory disturbances. At night, the apnoea-hypopnoea index (AHI) was 11 +/- 6. The patients with more symptoms during the daytime had the highest AHI scores. Most of the apnoeas (85%) were central, particularly during rapid eye movement (REM) sleep. Sleep architecture was well-preserved. Arterial desaturation (> 5% below baseline) occurred during 25 +/- 23% of total time. AHI correlated with daytime PaO2, and AHI in REM sleep correlated with age. A stepwise multivariate analysis showed that PaO2 and, to some extent, the degree of airflow obstruction were significantly correlated with AHI. We conclude that sleep-related respiratory disturbance are frequently present in patients with Duchenne muscular dystrophy. Therefore, physicians should look for symptoms related to sleep-related respiratory disturbances in these patients.(ABSTRACT TRUNCATED AT 250 WORDS)

This article has been cited by other articles:

				M. Toussaint, M. Chatwin, and P. Soudon Review Article: Mechanical ventilation in Duchenne patients with chronic respiratory insufficiency: clinical implications of 20 years published experience Chronic Respiratory Disease, August 1, 2007; 4(3): 167 - 177. [Abstract] [PDF]

				A. K. Simonds Recent Advances in Respiratory Care for Neuromuscular Disease Chest, December 1, 2006; 130(6): 1879 - 1886. [Abstract] [Full Text] [PDF]

				F. Lofaso, D. Orlikowski, and J-C. Raphael Ventilatory assistance in patients with Duchenne muscular dystrophy. Eur. Respir. J., September 1, 2006; 28(3): 468 - 469. [Full Text] [PDF]

				R Ragette, U Mellies, C Schwake, T Voit, and H Teschler Patterns and predictors of sleep disordered breathing in primary myopathies Thorax, August 1, 2002; 57(8): 724 - 728. [Abstract] [Full Text] [PDF]

				S.C. Bourke and G.J. Gibson Sleep and breathing in neuromuscular disease Eur. Respir. J., June 1, 2002; 19(6): 1194 - 1201. [Abstract] [Full Text] [PDF]

				M. F. PHILLIPS, R. C. M. QUINLIVAN, R. H. T. EDWARDS, and P. M. A. CALVERLEY Changes in Spirometry Over Time as a Prognostic Marker in Patients with Duchenne Muscular Dystrophy Am. J. Respir. Crit. Care Med., December 15, 2001; 164(12): 2191 - 2194. [Abstract] [Full Text] [PDF]

				S.C. Bourke, P.J. Shaw, and G.J. Gibson Respiratory function vs sleep-disordered breathing as predictors of QOL in ALS Neurology, December 11, 2001; 57(11): 2040 - 2044. [Abstract] [Full Text] [PDF]

				C. L. MARCUS Sleep-disordered Breathing in Children Am. J. Respir. Crit. Care Med., July 1, 2001; 164(1): 16 - 30. [Full Text] [PDF]

				P. ATTAL, F. LAMBERT, S. MARCHAND-ADAM, S. BOBIN, J.-C. POURNY, D. CHEMLA, Y. LECARPENTIER, and C. COIRAULT Severe Mechanical Dysfunction in Pharyngeal Muscle from Adult mdx Mice Am. J. Respir. Crit. Care Med., July 1, 2000; 162(1): 278 - 281. [Abstract] [Full Text]

				C. A. HUKINS and D. R. HILLMAN Daytime Predictors of Sleep Hypoventilation in Duchenne Muscular Dystrophy Am. J. Respir. Crit. Care Med., January 1, 2000; 161(1): 166 - 170. [Abstract] [Full Text]