Patients with proven neuromuscular disorders known to be potentially associated with significant diaphragmatic weakness, e. g. ALS, myotonic dystrophy type 1, limb-girdle muscular dystrophy, Duchenne and Becker muscular dystrophy. Patients already receiving home ventilatory support will not be included in the study.

Patient with proven obstructive sleep apnea syndrome prior to CPAP initiation.

Patients with sleep disorders other than sleep-related breathing disorders, e. g. narcolepsy, hypersomnia, parasomnia or sleep-related movement disorders.

Neuromuscular Disorders

All patients answer selected questionnaires on sleep-related symptoms, sleep quality, daytime performance, and health-related quality of life.

patient-filled questionnaires

Measurement of forced vital capacity, maximum inspiratory pressure and maximum expiratory pressure

spiromanometry

University Hospital Münster, Department of Sleep Medicine and Neuromuscular Disorders

Bethanien Hospital

Screening Questionnaire for Respiratory Muscle Weakness and Sleep-disordered Breathing in Neuromuscular Disorders

Validation of a German Language Screening Questionnaire for Symptoms of Respiratory Muscle Weakness and Sleep-disordered Breathing in Patients With Neuromuscular Disorders

Matthias Boentert

MD, Senior Consultant in Neurology and Sleep Medicine

Westfälische Wilhelms-Universität Münster

Arm assigned to AVAPS mode for nocturnal ventilation with the same setting than bilevel pressure support but with AVAPS mode activated

Arm treated only with bilevel pressure support for nocturnal ventilation without activation of AVAPS mode

Myopathy

Home ventilation only the mode AVAPS will be used if the patient is randomized in the experimental group.

Nocturnal ventilation

Home ventilation Unit , Raymond Poincaré hospital

Efficacy and Tolerance of AVAPS Mode in Myotonic Dystrophy

Prospective Randomized Controlled Single Blind Study of Efficacy and Tolerance of AVAPS Mode Compared to Bilevel Pressure Ventilation un Adult Patients With Myotonic Dystrophy

To evaluate efficacy of AVAPS Mode versus bilevel pressure mode at day 7 on arterial PCO2 under ventilation after launching ventilation

To evaluate efficacy of AVAPS Mode at day 90 on daytime arterial PCO2 after launching ventilation.

To evaluate compliance (h/24h) to ventilation at days 7 and 90.

To evaluate clinical efficacy on respiratory symptoms, dyspnea and sleepiness at day 1 and 90.

To evaluate effect of AVAPS on polysomnography, nocturnal SaO2, nocturnal PtCO2 at day 90.

To evaluate Multiple sleep latency and Maintenance of wakefulness tests at day 90.

To evaluate effect of AVAPS on respiratory parameters VC and mouth maximal pressures.

myotonic dystrophy/céphalée

Screening Questionnaire for Respiratory Muscle Weakness and Sleep-disordered Breathing in Neuromuscular Disorders

Efficacy and Tolerance of AVAPS Mode in Myotonic Dystrophy

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