Objective: The purpose of this study was to assess sleep and breathing
in patients with amyotrophic lateral sclerosis (ALS) with bulbar musc
le involvement. Design: Prospective, controlled study of sleep and bre
athing measured during polysomnography. Setting: University teaching h
ospital and referral center, Patients: Patients with definite ALS and
healthy age-matched control subjects.Interventions: Eighteen ALS patie
nts and 10 age-matched control subjects underwent one night of polysom
nography, Thirteen patients with ALS were studied for a second night.
Results: The ALS patients had more arousals per hour (p=0.008), more s
tage 1 sleep (p=0.01), and a shorter total sleep time (TST) (279+/-69
vs 331.4+/-55.9 min, mean+/-SD, p=0.04) than the control subjects. The
ALS patients had mild sleep-disordered breathing with a greater apnea
/hypopnea index (AHI) than the control subjects (p=0.005). On the seco
nd night of polysomnography, there was an increase in TST (p=0.003) an
d rapid eye movement (REM) sleep (p=0.009), an improvement in sleep ef
ficiency (p=0.02), and less stage 1 sleep (p=0.04). Eight ALS patients
had sleep-disordered breathing consisting of periods of hypoventilati
on, predominantly during REM sleep. Conclusions: Sleep-disordered brea
thing occurs in patients with ALS and is similar to patients without A
LS with respiratory muscle weakness. No obstructive sleep apnea was ob
served. One potential reason for its absence might be the inability of
patients with respiratory muscle weakness to generate an inspiratory
pressure greater than the upper airway closing pressure. This hypothes
is should be addressed in future studies.