Matamala José Manuel, Moreno-Roco Javier, Acosta Ignacio, Hughes Ricardo, Lillo Patricia, Casar Juan Carlos, Earle Nicholas
Laboratorio de Neurología y Neurofisiología Traslacional, Facultad de Medicina, Universidad de Chile, Santiago, Chile.
Departamento de Neurología y Neurocirugía, Hospital Clínico de, Universidad de Chile, Santiago, Chile.
Rev Med Chil. 2022 Dec;150(12):1633-1646. doi: 10.4067/s0034-98872022001201633.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that mainly affects the motor system, resulting in progressive weakness and muscle wasting. Despite the tremendous advances in physiopathological and clinical characterization, we do not have a curative treatment yet. The progressive and fatal course of ALS makes its management particularly complex and challenging given the diversity of symptoms presenting during the disease progression. The main goal in the treatment of ALS patients is to minimize morbidity and maximize the quality of life. Currently, a series of therapeutic interventions improve the quality of life and prolong survival, including multidisciplinary care, respiratory management, and disease-modifying therapy. Within the supportive interventions, weight maintenance through nutritional and metabolic support is critical. In addition, the management of neuropsychiatric manifestations and preservation of communicative capacity before speech loss are also crucial. Lastly, early palliative care intervention is essential to optimize symptomatic management. Anticipatory guidelines to face the inevitable patient deterioration should be devised. This article updates the main therapeutic strategies used in these patients, including evolving clinical trials with promising novel therapies.
肌萎缩侧索硬化症(ALS)是一种主要影响运动系统的神经退行性疾病,会导致进行性肌无力和肌肉萎缩。尽管在生理病理学和临床特征方面取得了巨大进展,但我们尚未找到治愈方法。ALS的进行性和致命病程使其管理特别复杂且具有挑战性,因为疾病进展过程中会出现多种症状。治疗ALS患者的主要目标是将发病率降至最低,并最大限度地提高生活质量。目前,一系列治疗干预措施可改善生活质量并延长生存期,包括多学科护理、呼吸管理和疾病修饰治疗。在支持性干预措施中,通过营养和代谢支持维持体重至关重要。此外,神经精神症状的管理以及在言语丧失前保留沟通能力也很关键。最后,早期姑息治疗干预对于优化症状管理至关重要。应制定应对患者不可避免恶化的前瞻性指南。本文更新了这些患者使用的主要治疗策略,包括开展中的有前景新疗法的临床试验。