Multi-Center, Randomized Controlled Study of the NeuRx® Diaphragm Pacing System™ (DPS)In Participants With Amyotrophic Lateral Sclerosis (ALS)

Study Purpose:

The study is being conducted to determine if DPS treatment for people with ALS and hypoventilation is associated with improved survival or diaphragm function.

The primary objective of the study is to conduct a multi-center, randomized controlled clinical trial comparing standard of care (control) to diaphragm stimulator treatment with the NeuRx® Diaphragm Pacing System™ (DPS) with respect to survival.

The secondary objective of the study is to conduct a multi-center, randomized controlled clinical trial to compare standard of care treatment (control) to DPS in ALS subjects with hypoventilation.

Study Status:

Not recruiting


Amyotrophic Lateral Sclerosis (ALS)

Study Type:


Type of Intervention:


Intervention Name:

NeuRx® Diaphragm Pacing System™ (DPS)



Phase 2

Study Chair(s)/Principal Investigator(s):

Jeremy Shefner, MD, PhD, Barrow Neurological Institute, Jonathan Katz, MD, California Pacific Medical Center ID:


Neals Affiliated?


Coordinating Center Contact Information

Massachusetts General Hospital

165 Cambridge Street
Boston, Massachusetts, 02114 United States

Full Study Summary:

The study is a randomized controlled study to compare standard of care (control) to DPS (diaphragm stimulator named The NeuRx® Diaphragm Pacing System™). Although it remains uncertain if DPS is efficacious for the ALS subject, given the preliminary studies performed by the device manufacturer, it has received FDA humanitarian device exemption (HDE) approval as a humanitarian use device (HUD). This study will utilize a 2:1 randomization schedule such that eligible ALS subjects will have a 2/3 chance of receiving DPS and 1/3 chance of standard of care (control) treatment.

The NeuRx® Diaphragm Pacing System™ (DPS) is a four channel, implanted percutaneous diaphragm muscle stimulation system. Under general anesthesia, the intramuscular electrodes are laparoscopically implanted in the diaphragm. The ends of the implanted electrodes are tunneled subcutaneously to an exit site on the chest or abdominal wall and connected to an external stimulator.

Study Sponsor:

Barrow Neurological Institute

Participant Duration:

Subjects will take part in the study for approximately 18 months. Subjects will have 6 in-person visits and monthly phone visits during the study.

Estimated Enrollment:


Estimated Study Start Date:

07 / 31 / 2013

Estimated Study Completion Date:

04 / 01 / 2017

Posting Last Modified Date:

03 / 09 / 2023

Date Study Added to

09 / 10 / 2013

Minimum Age:

21 Years

Maximum Age:


Can participants use Riluzole?


Inclusion Criteria:

1. Age 21 years or older.

2. Sporadic or familial ALS diagnosed as definite, probable or possible ALS as defined by revised El Escorial criteria.

3. Evidence of hypoventilation at Screening with at least one of the following:

1. Maximal static inspiratory pressure (MIP) <60 cm H20.

2. Upright or supine forced vital capacity (FVC) <50% predicted for gender, age, and height.

4. A phrenic nerve potential should be recordable bilaterally.

5. Subjects must not have taken riluzole for at least 30 days, or be on a stable dose of riluzole for at least 30 days, prior to randomization (riluzole-naïve subjects are permitted in the study).

6. Capable of providing informed consent and following trial procedures.

7. Geographically accessible to the site.

8. Negative urine pregnancy test at Screening in women of child bearing potential (WOCBP). (Women who are post-menopausal or who have had a hysterectomy are deemed not of child bearing potential).

9. Women of child bearing potential must use an adequate form of contraception: abstinence, hormonal contraception (oral contraception, implanted contraception, injected contraception or other hormonal (patch or contraceptive ring, for example) contraception), intrauterine device (IUD) in place for ≥ 3 months, barrier method in conjunction with spermicide, or another adequate method.

Exclusion Criteria:

1. Upright forced vital capacity (FVC) ≤ 45% of predicted for gender, age, and height.

2. Any prior use of non-invasive ventilation (NIV) outside of sleep (nighttime or naps).

3. Any pulmonary or cardiac disorder or other medical disorder that would be a contraindication for general anesthesia or DPS hardware implantation in the chest.

4. Implanted electrical device such as a pacemaker or cardiac defibrillator.

5. Known diaphragm abnormality such as hiatal hernia or para-esophageal hernia of abdominal contents into the thoracic cavity.

6. Participation in another treatment research study for people with ALS.

7. Exposure to any other agent currently under investigation for the treatment of people with ALS (off-label use or investigational) within 30 days of the Screening Visit.

8. Clinically significant history of unstable or severe cardiac, oncologic, hepatic, psychiatric, renal disease, or other medically significant illness.

9. Pregnant women or women currently breastfeeding.

Barrow Neurological Institute

Phoenix, Arizona
United States

Cedars-Sinai Medical Center

Los Angeles, California
United States

California Pacific Medical Center

San Diego, California
United States

Stanford University

Stanford, California
United States

Hospital for Special Care

New Britain, Connecticut
United States

Mayo Clinic Florida

Jacksonville, Florida
United States

University of Florida, Jacksonville

Jacksonville, Florida
United States

University of Iowa

Iowa City, Iowa
United States

Massachusetts General Hospital

Boston, Massachusetts
United States

Henry Ford Health Systems

Detroit, Michigan
United States

St Louis University

Saint Louis, Missouri
United States

Neurology Associates P.C.

Lincoln, Nebraska
United States

Hospital for Special Surgery

New York, New York
United States

SUNY Upstate Medical University

Syracuse, New York
United States

Carolinas Health Care

Charlotte, North Carolina
United States

Wake Forest University

Winston-Salem, North Carolina
United States

Cleveland Clinic

Cleveland, Ohio
United States

Ohio State University

Columbus, Ohio
United States

Providence ALS Center

Portland, Oregon
United States

Drexel University

Philadelphia, Pennsylvania
United States

University of Pittsburgh

Pittsburgh, Pennsylvania
United States

University of Texas Southwestern

Dallas, Texas
United States

University of Texas

San Antonio, Texas
United States

University of Vermont

Burlington, Vermont
United States

Virginia Mason Medical Center

Seattle, Washington
United States