No participants enrolled

The purpose of this research study is to collect more information about the use, safety, and effectiveness of the NeuRx DPS® in ALS patients.

Characterize the safety and benefit of on-label Humanitarian Device Exemption (HDE) use of DPS in patients with ALS who initiated noninvasive ventilation (NIV).

Inclusion Criteria:

• Age 21 or older

• Familial or sporadic ALS diagnosed as possible, laboratory-supported probable, probable, or definite according to the World Federation of Neurology El Escorial criteria

• Bilateral phrenic nerve function clinically acceptable as demonstrated by bilateral diaphragm movement with fluoroscopic sniff test or with phrenic nerve conduction studies

• Chronic hypoventilation was documented by at least one of the following:

  • MIP (respiratory muscle strength) less than 60 cmH2O, or
  • Forced Vital Capacity (FVC) less than 50% predicted, or
  • PaCO2 greater than or equal to 45 mmHg, or
  • Nocturnal SaO2 (arterial oxygen saturation) less than or equal to 88% for at least five continuous minutes

• Initiation of non-invasive ventilation at or before time of enrollment

• Suitable surgical candidate to receive diaphragm pacing stimulation

• Negative pregnancy test in female participants of childbearing potential (treatment group)

• Informed consent from patient or designated representative

Exclusion Criteria:

• FVC less than 45% predicted within 10 days prior to surgery.
• Participation in simultaneous therapeutic clinical trial which could affect the safety or benefit outcomes of this study.

Open label