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.
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)
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