Neuromuscular diseases are frequently associated with respiratory failure, which requires Non Invasive Ventilation (NIV). Currently, the NIV installation is done during an hospitalization of several days. This hospitalization is problematic because of availability of beds, logistical difficulties for the patient and estrangement from the usual environment.

For this reasons, the NIV installation at home could be an interesting alternative for both the patient and the medical staff.

The aim of this pilot study is to test the feasibility of NIV installation at home, using telemedicine as a remote monitoring tool, and to assess its impact on the ventilation compliance.

For the study protocol, patients will be hospitalized for the first day to define the ventilation mode and initial parameters, and to choose the best adapted interface.

Then, the patient is discharged from hospital and parameters adaptation is continued at home during the following five days by the hospital medical staff, using telemedicine. An ASV Santé employee visits the patient at home everyday during 5 days to ensure follow-up.

Inclusion Criteria:

• Man or Women aged over 18 years
• Neuromuscular disease or kyphoscoliosis
• At least one sign among : dyspnea, orthopnea, daytime sleepiness, cephalalgia, asthenia
• At least one sign among : hypercapnia > 45 mmHg, desaturation time < 88%, night desaturation time > 5 min, Vital Capacity < 60% or Pimax < 60 cm H2O.

Exclusion Criteria:

• Mechanic ventilation refusal
• Patient living alone
• Acute respiratory failure
• Patient who need a third party for the ventilation installation
• Severe respiratory limitation
• Home oxygen