title: Sleep disorders and diaphragmatic function in patients with amyotrophic lateral sclerosis.
abstract: 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).
authors.affiliation: Service de Pneumologie, UPRES EA 2397, Explorations Fonctionnelles Respiratoires, Neurologie et Explorations Fonctionnelles du Systeme Nerveux, Groupe Hospitalier Pitie-Salpetriere, Paris, France. isabelle.arnulf@psl.ap-hop-paris.fr
authors.forename: I
authors.initials: I
authors.lastname: Arnulf
authors.forename: T
authors.initials: T
authors.lastname: Similowski
authors.forename: F
authors.initials: F
authors.lastname: Salachas
authors.forename: L
authors.initials: L
authors.lastname: Garma
authors.forename: S
authors.initials: S
authors.lastname: Mehiri
authors.forename: V
authors.initials: V
authors.lastname: Attali
authors.forename: V
authors.initials: V
authors.lastname: Behin-Bellhesen
authors.forename: V
authors.initials: V
authors.lastname: Meininger
authors.forename: J P
authors.initials: JP
authors.lastname: Derenne
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003964:Diaphragm / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology*; D009046:Motor Neurons / Q000502:physiology; D017286:Polysomnography; D011379:Prognosis; D012133:Respiratory Paralysis / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology*; D012891:Sleep Apnea Syndromes / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology*; D015996:Survival Rate