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<a href="http://doi.org/10.1136/jnnp.65.2.225" target="_blank" rel="noreferrer">http://doi.org/10.1136/jnnp.65.2.225</a>
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Title
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Sleep and neuromuscular disease: bilevel positive airway pressure by nasal mask as a treatment for sleep disordered breathing in patients with neuromuscular disease
Publisher
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Journal Of Neurology, Neurosurgery, And Psychiatry
Date
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1998
Subject
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Child; Female; Humans; Male; Adult; Follow-Up Studies; Middle Aged; Positive-Pressure Respiration; Treatment Outcome; adolescent; Q3 Literature Search; Intermittent Positive-Pressure Ventilation; Lung Volume Measurements; Masks; Muscular Dystrophies/physiopathology/therapy; Neuromuscular Diseases/physiopathology/therapy; Polysomnography; Pulmonary Ventilation/physiology; Sleep Apnea Syndromes/physiopathology/therapy
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Guilleminault C; Philip P; Robinson A
Description
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OBJECTIVE: Investigation of the therapeutic effects of bilevel positive airway pressure delivered by nasal mask in patients with neuromuscular disease. METHODS: 20 patients with neuromuscular disease were evaluated for symptoms of nocturnal sleep disruption. These symptoms included daytime tiredness, fatigue, sleepiness, and complaints of insomnia. The patients were studied with nocturnal polysomnograms and daytime multiple sleep latency tests (MSLT). Their immediate and long term responses to bilevel positive airway pressure were also investigated. The study took place at the Stanford University Sleep Disorders Clinic. Some of the polygraphic evaluations were performed with portable equipment in the patients' homes. The reported population comprised 20 patients, all of whom had progressive neuromuscular disease. Five of the patients were women. Four patients had muscular dystrophy, six had myotonic dystrophy, and two patients each had mitochondrial myopathy and glycogen storage disease. Two patients had post-traumatic lesions, one bulbar and the other 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 of these were initially maintained on bilevel positive airway pressure spontaneous (S) mode, although one patient required a switch to the timed (T) mode within a year. The mean expiratory positive airway pressure (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 < or = 8 minutes in 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 the mean 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/min bled into their masks. Three patients with myotonic dystrophy presented continued daytime somnolence despite apparent adequate treatment of their sleep disordered breathing. This required the addition of stimulant medication to their regimen. During this time three additional subjects had to be switched to nasal mask intermittent positive pressure ventilation delivered by traditional volume cycled home ventilator (volume controlled NIPPV). CONCLUSIONS: Bilevel positive airway pressure delivered by nasal mask may be used successfully to treat sleep disordered breathing associated with neuromuscular disease. This device can be employed to assist nocturnal ventilation by either the spontaneous or timed mode. In the United States it is less expensive and easier to institute than volume controlled NIPPV and may be as efficacious as this mode if close surveillance and regular reevaluation of the patient's status is maintained.
1998
Identifier
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<a href="http://doi.org/10.1136/jnnp.65.2.225" target="_blank" rel="noreferrer">10.1136/jnnp.65.2.225</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Type
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Journal Article
1998
Adolescent
Adult
Backlog
Child
Female
Follow-up Studies
Guilleminault C
Humans
Intermittent Positive-Pressure Ventilation
Journal Article
Journal Of Neurology, Neurosurgery, And Psychiatry
Lung Volume Measurements
Male
Masks
Middle Aged
Muscular Dystrophies/physiopathology/therapy
Neuromuscular Diseases/physiopathology/therapy
Philip P
Polysomnography
Positive-Pressure Respiration
Pulmonary Ventilation/physiology
Q3 Scoping Review Results
Robinson A
Sleep Apnea Syndromes/physiopathology/therapy
Treatment Outcome