Am. J. Respir. Crit. Care Med., Volume 161, Number 3, March 2000, 849-856

Sleep Disorders and Diaphragmatic Function in Patients with Amyotrophic Lateral Sclerosis

ISABELLE ARNULF, THOMAS SIMILOWSKI, FRANÇOIS SALACHAS, LUCILE GARMA, SELMA MEHIRI, VALERIE ATTALI, VALERIE BEHIN-BELLHESEN, VINCENT MEININGER, and JEAN-PHILIPPE DERENNE

Services de Pneumologie, UPRES EA 2397, Explorations Fonctionnelles Respiratoires, Neurologie et Explorations Fonctionnelles du Systeme Nerveux, Groupe Hospitalier Pitie-Salpetriere, Paris, France

In amyotrophic lateral sclerosis (ALS), the progressive loss of upper and lower motor neurons leads to respiratory failure, often with predominant diaphragm dysfunction, and death. Because the diaphragm is the only active inspiratory muscle during rapid eye movement (REM) sleep, there is a high theoretical risk of respiratory disorders during REM sleep in patients with ALS. To assess this hypothesis, we studied sleep characteristics (polysomnography) in 21 patients with ALS, stratified according to the presence or absence of diaphragmatic dysfunction. Diaphragmatic dysfunction was defined as an absent or delayed diaphragm response to cervical or cortical magnetic stimulation, abdominal paradox, or respiratory pulse (Group 1, 13 patients). These patients did not differ in age, clinical course, or form (bulbar or spinal) from the eight others, who did not have diaphragmatic dysfunction (Group 2). REM sleep was reduced in Group 1 (7 ± 7% of total sleep time; mean ± SD) and normal in Group 2 (18 ± 6%, p = 0.004). Apneas or hypopneas were rare in both groups. In Group 1, REM sleep was absent or minimal (less than 3 min) in five patients. An unusual and remarkable preservation of phasic inspiratory sternomastoid activation during REM was associated with longer REM sleep duration in six of the other patients with diaphragmatic dysfunction. Median survival time was dramatically shorter (217 d) in Group 1 than in Group 2 (619 d, p = 0.015). Arnulf I, Similowski T, Salachas F, Garma L, Mehiri S, Attali V, Behin-Bellhesen V, Meininger V, Derenne J-P. Sleep disorders and diaphragmatic function in patients with amyotrophic lateral sclerosis.

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