2017 年 34 巻 3 号 p. 205-208
Respiratory muscle paralysis is a hallmark of amyotrophic lateral sclerosis (ALS). Respiratory dysfunction in ALS should be appropriately predicted, evaluated, and managed with the goal of improving the patient's quality of life. Voice changes, anorexia, and nocturnal sleep deprivation may be early signs of respiratory decline. Forced vital capacity, peak cough flow, examination for nocturnal desaturation or hypoventilation, and phrenic nerve conduction study are useful as parts of a routine examination for assessing respiratory function. Noninvasive (NIV) and tracheostomy–invasive ventilation (TIV) are the most effective methods for improving patients' survival rate and quality of life. NIV prolongs the survival period for about one year and TIV for more than ten years. Clinicians should fully inform patients of the advantages and disadvantages of NIV and TIV at an early stage of the disease. If a patient elects not to use a ventilator, terminal and palliative care are needed. The important points to consider for the palliation of respiratory distress are (1) sufficient oxygenation, (2) preserving airway patency, and (3) drug therapy including use of opioids. Opioids in particular, which are still unfamiliar to Japanese neurologists, are crucial to successful palliative therapy in ALS patients.
