2019 年 36 巻 4 号 p. 337-340
Patients with amyotrophic lateral sclerosis (ALS) develop progressive generalized skeletal muscle atrophy, weakness, and die on average 3–4 years from onset or require permanent ventilation. Even now, we have no curative treatment for ALS, but there is much to do in medical care. As standard care, we will administer progression inhibitors, manage nutritional and respiratory status, provide symptomatic treatments, introduce rehabilitation, support communication disorder and build a multidisciplinary support team. The clinical courses and prognoses of ALS patients are shown to be very diverse, and the treatment and care plan for each patient should be determined according to the status and progress of the individual patient.
The development of disease–modifying treatments that alter the pathophysiology of neurodegeneration in ALS patients is being vigorously carried out. The background is that many genes and molecules related to the pathogenesis of ALS have been identified since the 2000s. Therapeutic development of ALS is approaching the stage of developing disease–modifying therapy by elucidating pathogenic genes and molecular pathogenesis. There has been great progress in elucidating the molecular pathogenesis of ALS, and it is expected to become an active area of drug discovery.
