Contemporary Standards of Care in Amyotrophic Lateral Sclerosis: A Comprehensive Review of Clinical Guidelines and Evidence-Based Practice

Amyotrophic Lateral Sclerosis is a progressive neurodegenerative disease that affects motor neurons, causing muscle weakness, dysphagia, and respiratory failure. Due to its clinical complexity and wide-ranging impact on quality of life, the management of patients with ALS requires an integrated multidisciplinary approach. The European Academy of Neurology in collaboration with the European Reference Network for Neuromuscular Diseases (ERN Euro-NMD) has published the latest guidelines (92024) for the management of ALS, which are based on scientific evidence and the GRADE methodology. A narrative literature review was conducted using peer-reviewed articles, clinical practice guidelines, systematic reviews, model treatments and randomized controlled published between 2015-2025. Databases researched included PubMed, Scopus and CINAHL. Preference was given to studies addressing evidence-based management and treatment models of care in ALS. Riluzole remains the standard therapy for all patients from diagnosis. Tofersen is recommended for patients with ALS associated with SOD1 mutation. Edavarone and AMS0035 are not recommended for routine use due to insufficient evidence. Multidisciplinary care increases survival and improves quality of life. Nutritional support and non-invasive ventilation are the mainstays of case management. Management of symptoms (spasticity, cramps, sialorrhea, fatigue, emotional lability) is essential for patient comfort. All patients with ALS should start treatment with riluzole from diagnosis. Patients with SOD1 mutation should be evaluated for treatment with tofersen. Other treatments (edavarone, AMX0035, stem cells) should not be used outside of research protocols. Multidisciplinary care should be standard, with regular visits every 2-3 months. Early assessment for dysphagia and planning for gastrostomy where necessary. Monitoring of respiratory function and provision of non-invasive ventilation in a timely manner. Management of symptoms with pharmacological approaches as needed. Early and ongoing discussion of patient preferences regarding treatments and end-of-life care.