Lung Insufflation Capacity Training and Respiratory Function in Amyotrophic Lateral Sclerosis

NCT07257302 | Recruiting

• 2 other identifiers: NCNP-LIC-ALS-202525K14423
• Study Type: interventional
• Target: 15
• Locations: 1 country
• Sites: 1

Brief Summary

The goal of this clinical trial is to learn whether lung insufflation capacity (LIC) training can help maintain respiratory function and prolong survival in people with amyotrophic lateral sclerosis (ALS). The main questions it aims to answer are:

• Does early and continuous LIC training slow the decline in forced vital capacity (FVC)?
• Does LIC training delay the need for tracheostomy or noninvasive ventilation (NIV)? This single-center study at the National Center of Neurology and Psychiatry (NCNP) in Japan will enroll 15 adults with ALS, diagnosed according to the El Escorial or Awaji criteria. Participants will:
• Use the LIC Trainer device to perform lung insufflation training twice daily at home
• Visit the clinic every 3 months for respiratory and functional assessments
• Have lung tests, including FVC, LIC, maximum insufflation capacity (MIC), cough peak flow (CPF), and complete the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) There is no control group within this trial; researchers will compare results with matched historical controls from the Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) database.

Conditions

Amyotrophic Lateral Sclerosis (ALS)

Keywords

LIC Trainer; lung volume recruitment; respiratory rehabilitation; FVC; ALS; ALSFRS-R; cough peak flow

Outcome Measures

Primary Outcomes (1)

• Overall survival (time to tracheostomy or death)

  Overall survival is defined as the time from the initiation of lung insufflation capacity (LIC) training to tracheostomy or death, whichever occurs first. Participants who remain alive without tracheostomy at the end of follow-up will be censored at their last known assessment. Survival analysis will use Kaplan-Meier estimates and Cox proportional hazards models. The outcome will also be compared with matched historical controls from the PRO-ACT ALS database using propensity score matching.

  From the start of LIC training (Day 0) until tracheostomy or death, up to 36 months

Secondary Outcomes (5)

• Change in forced vital capacity (%FVC)

  Baseline and every 3 months for up to 36 months

• Change in lung insufflation capacity (LIC) and maximum insufflation capacity (MIC)

  Baseline and every 3 months for up to 36 months

• Change in cough peak flow (CPF) and assisted CPF

  Baseline and every 3 months for up to 36 months

• Change in ALS Functional Rating Scale-Revised (ALSFRS-R) score

  Baseline and every 3 months for up to 36 months

• Incidence of respiratory infections

  Up to 36 months

Study Arms (1)

LIC Training

EXPERIMENTAL

Participants with amyotrophic lateral sclerosis (ALS) will perform lung insufflation capacity (LIC) training using the LIC Trainer device to maintain lung and chest wall compliance. Each session consists of about 10 assisted insufflations performed twice daily at home. Physical therapists provide initial instruction and re-evaluation every 3 months during the 36-month follow-up period. The intervention aims to preserve pulmonary function, reduce respiratory complications, and prolong survival.

Device: LIC Trainer (Lung Insufflation Capacity Trainer)

Interventions

LIC Trainer (Lung Insufflation Capacity Trainer) DEVICE

The LIC Trainer is a medical device designed to enable lung insufflation through a one-way valve system, allowing passive air stacking without voluntary glottic closure. The device includes a safety valve that automatically releases pressure above 60 cmH₂O and a manual relief valve to ensure patient safety. It is used with a bag-valve-mask to deliver multiple assisted insufflations, approximately 10 per session, twice daily. Participants with amyotrophic lateral sclerosis (ALS) perform LIC training at home after initial instruction by physical therapists and are re-evaluated every 3 months for up to 36 months.

Also known as: Lung Insufflation Capacity Trainer, LIC training, air stacking training

LIC Training

Eligibility Criteria

• Age: 20 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Age 20 years or older
• Diagnosis of amyotrophic lateral sclerosis (ALS) according to the El Escorial or Awaji criteria
• Not using noninvasive ventilation (NIV) and without tracheostomy at the start of LIC training
• Able to perform study assessments and provide written informed consent (or assisted signature with communication aid)

You may not qualify if:

• Chronic pulmonary disease other than ALS (e.g., COPD, interstitial lung disease)
• Severe cognitive or communication impairment preventing study participation
• Uncontrolled cardiovascular disease, including unstable angina, recent myocardial infarction, decompensated heart failure, serious arrhythmia, severe aortic stenosis, or active myocarditis/endocarditis
• Uncontrolled hypertension
• Acute systemic illness or fever
• Recent pulmonary embolism, acute cor pulmonale, or severe pulmonary hypertension
• Severe hepatic or renal dysfunction
• Any condition judged inappropriate by the investigator

Sponsors & Collaborators

• National Center of Neurology and Psychiatry, Japan: lead

Study Sites (1)

National Center of Neurology and Psychiatry (NCNP)

Kodaira, Tokyo, 187-8551, Japan

RECRUITING

Related Links

• Public registration record in the UMIN Clinical Trials Registry (Japan)

MeSH Terms

Conditions

Amyotrophic Lateral Sclerosis

Condition Hierarchy (Ancestors)

Spinal Cord Diseases; Central Nervous System Diseases; Nervous System Diseases; Motor Neuron Disease; Neurodegenerative Diseases; TDP-43 Proteinopathies; Neuromuscular Diseases; Proteostasis Deficiencies; Metabolic Diseases; Nutritional and Metabolic Diseases

Study Officials

• Taiyo Kawaguchi, MD

  National Center of Neurology and Psychiatry

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Study Office

CONTACT

+81-42-341-2712; rinri-jimu@ncnp.go.jp

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Masking Details: All participants and investigators are aware of the intervention. No blinding is applied.
• Purpose: TREATMENT
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Physician, Department of Rehabilitation Medicine

Model Details: Single-arm prospective study comparing pre- and post-intervention changes in respiratory function among patients with ALS receiving LIC training.

Study Record Dates

• First Submitted: November 20, 2025
• First Posted: December 2, 2025
• Study Start: November 1, 2025
• Primary Completion (Estimated): March 31, 2028
• Study Completion (Estimated): March 31, 2030
• Last Updated: December 9, 2025

Record last verified: 2025-11

Data Sharing

• IPD Sharing: Will not share

Locations

JP (1)