Sleep and neuromuscular disease: bilevel positive airway pressure by nasal mask as a treatment for sleep disordered breathing in patients with neuromuscular disease

摘要

OBJECTIVE—Investigationof the therapeutic effects of bilevel positive airway pressuredelivered by nasal mask in patients with neuromuscular disease.
METHODS—20 patientswith neuromuscular disease were evaluated for symptoms of nocturnalsleep disruption. These symptoms included daytime tiredness, fatigue,sleepiness, and complaints of insomnia. The patients were studied withnocturnal polysomnograms and daytime multiple sleep latency tests(MSLT). Their immediate and long term responses to bilevel positiveairway pressure were also investigated. The study took place at theStanford University Sleep Disorders Clinic. Some of the polygraphicevaluations were performed with portable equipment in the patients'homes. The reported population comprised 20 patients, all of whom hadprogressive neuromuscular disease. Five of the patients were women.Four patients had muscular dystrophy, six had myotonic dystrophy, andtwo patients each had mitochondrial myopathy and glycogen storagedisease. Two patients had post-traumatic lesions, one bulbar and theother phrenic. The remaining patients had vascular myopathy,unclassified myopathy, syringomyelia, and slow evolving spinocerebellar degeneration.
RESULTS—19 of the 20 patients accepted some form of non-invasive ventilation. All but one ofthese were initially maintained on bilevel positive airway pressurespontaneous (S) mode, although one patient required a switch to thetimed (T) mode within a year. The mean expiratory positive airwaypressure (EPAP) used was 4.5 with a range of 4 to 5 cm H2O.The mean inspiratory positive airway pressure (IPAP) was 11.5, range 9 to 14 cm H2O. Before treatment the MSLTs were ⩽ 8 minutesin 11 of the patients. The overall mean score was 8.2 (SD) 1.3 minutes.After long term treatment the mean MSLT was 12.5 (SD 2) minutes and themean ESS score was 7 (SD 3). During the mean 3.5 years of follow up,three patients needed supplemental oxygen at a flow of 0.5 to 1.0 l/minbled into their masks. Three patients with myotonic dystrophy presentedcontinued daytime somnolence despite apparent adequate treatment oftheir sleep disordered breathing. This required the addition ofstimulant medication to their regimen. During this time threeadditional subjects had to be switched to nasal mask intermittentpositive pressure ventilation delivered by traditional volume cycledhome ventilator (volume controlled NIPPV).
CONCLUSIONS—Bilevelpositive airway pressure delivered by nasal mask may be usedsuccessfully to treat sleep disordered breathing associated withneuromuscular disease. This device can be employed to assist nocturnalventilation by either the spontaneous or timed mode. In the UnitedStates it is less expensive and easier toinstitute than volume controlled NIPPV and may be as efficacious asthis mode if close surveillance and regular re-evaluation of thepatient's status is maintained.