Background: Progressive muscle weakness is aggravated not only in the skeletal muscles but also in the respiratory muscles in many patients with neuromuscular diseases (NMD). Inspiratory muscle training (IMT) has been reported as therapy for pulmonary rehabilitation to improve respiratory strength, endurance, exercise capacity, and quality of life, and to reduce dyspnea. Objects: The purpose of this study was to determine the effect of playing harmonica for 5 months on pulmonary function by assessing the force vital capacity (FVC), peak cough flow (PCF), maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and maximal voluntary ventilation (MVV) in patients with NMD. Methods: Six subjects with NMD participated in this study. The subjects played harmonica once a week for 2 hours at a harmonica academy and twice a week for 1 hour at home. Thus, training was performed thrice a week for 23 weeks. The examiner assessed pulmonary function by measuring FVC in the sitting and supine positions and PCF, MIP, MEP, and MVV in the sitting position at the beginning of training and once a month for 5 months. Results: Both sitting and supine FVC significantly increased after playing harmonica (p=.042), as did MIP (p=.043) and MEP (p=.042). Conclusion: Playing harmonica can be used as an effective method to improve pulmonary function in patients with NMD.

Respiratory distress resulting from a neuromuscular disorder is often misdiagnosed and not managed properly until development of severe respiratory failure. Amyotrophic lateral sclerosis (ALS) is a rare but archetypical motor neuron disease. Most cases manifest as weakness, muscular atrophy, and progressive paralysis of the limbs. Late stage of the disease is characterized by respiratory failure due to respiratory muscle weakness. In a few cases, 3% of patients, respiratory failure is the initial presentation, preceding other symptoms. We report on a case of ALS accompanied by acute respiratory failure and weaning failure, with a review of the literature.