Amyotrophic lateral sclerosis (ALS) is a progressive disease that involves the motor nervous system (including the cerebral motor cortex, motor nerve fiber conduction tracts, and brainstem motor nuclei, anterior horn cells), also known as motor neuron disease. The exact etiology of the disease is not yet known. Very cruelly, the survival period of patients with motor neuron disease after diagnosis is usually 2 to 5 years, and most ALS patients eventually die of progressive respiratory failure. To date, there is no effective way to cure or stop the progression of motor neuron disease. The search for drugs and/or interventions to prolong the survival and improve the quality of life of ALS patients has been ongoing since the 1980s. Unfortunately, the current status of drug therapy is not promising, and the only drug currently approved for ALS treatment, riluzole, only extends the survival of some ALS patients by about 3 months. Precisely because the most common cause of death in ALS is respiratory failure, respiratory assistance and management of respiratory function are very important. A growing body of research evidence suggests that a variety of noninvasive ventilation techniques have been used in the mid- or early stages of ALS that can preserve feeding and speech function, as well as somewhat prolong survival and slow the rate of decline in lung function in ALS patients, improve the overall quality of patient survival, and reduce the incidence of respiratory infections and their resulting health care costs. Respiratory muscle weakness occurs sooner or later in patients diagnosed with ALS and progresses continuously, although not at the same rate of deterioration. Respiratory muscle weakness can lead to hypoxia due to inadequate ventilation and carbon dioxide retention due to inadequate air exchange. The lack of oxygen in the patient’s blood and the hypercapnia (caused by carbon dioxide retention) cause the already damaged motor neurons to die or lose function more quickly, and the weakness and fatigue of the respiratory muscles innervated by the motor neurons becomes more pronounced, creating a vicious cycle. Chronic respiratory muscle fatigue also increases the risk of lung infections by weakening the cough reflex and making it difficult to expel secretions. Current medical opinion suggests that all ALS patients should be monitored for non-specific signs and symptoms of respiratory dysfunction, such as dyspnea after activity and lying down, inattention, nightmares – morning headache – excessive daytime sleepiness, and decreased chest breathing, and for periodic (recommended every three months) measurements of forceful spirometry. As soon as there is evidence of a decline in respiratory function, respiratory support therapy should be administered. The most used technique in ALS patients today is bi-level intermittent positive pressure ventilation (Bipap). This non-invasive breathing device is triggered by the patient’s own respiratory actions, which reduces respiratory muscle work and improves gas exchange, thus improving both sleep quality and compliance. Overall, the benefits of the Bipap are as follows: improved quality of life; prolonged survival; avoidance of endotracheal tube related infections and complications; reduced respiratory muscle fatigue; increased oxygenation and carbon dioxide removal; and no disruption of patient communication and diet. There are no detailed guidelines for setting and adjusting the parameters of the Bipap noninvasive ventilator, which are adjusted according to the individual patient’s stage of disease and tolerance level. Many studies suggest that patients with ALS who require prolonged noninvasive ventilation should use pressure triggering, which is more comfortable and compensates for air leaks. However, pressure triggering does not guarantee a minimum minute ventilation, and because many patients with respiratory muscle weakness during deep REM sleep experience a decrease in oxygen saturation, a mandatory assisted respiratory rate is required. The respiratory management of ALS patients is more standardized abroad, especially the use of Bipap in early patients. In the United States, non-invasive ventilator use in ALS patients has been reported to be between 49% and 67%, and some studies in Europe have also shown non-invasive ventilator use to be between 41% and 63%, while in Japan it has been reported to be around 50%. A literature has mentioned that the rate of non-invasive ventilator use in Taiwan is also 36%. A survey conducted by the Department of Neurology of the Third Hospital of Peking University showed that the use of ventilators in ALS patients in China was only about 10%, which is much lower than that in other countries and regions mentioned above. This is related to the economic conditions of patients in China and the fact that clinicians in China are not yet aware of the timing and significance of early use of non-invasive ventilators.