What are the treatment options for Amyotrophic Lateral Sclerosis (ALS)?

From the Guidelines

Amyotrophic lateral sclerosis (ALS) treatment should focus on managing symptoms and slowing disease progression through a multidisciplinary approach, including the use of FDA-approved medications such as riluzole and edaravone, as well as newer options like AMX0035, and prioritizing access to multidisciplinary care, services, and caregiver support, as recommended by the 2024 National Academies of Sciences, Engineering, and Medicine report 1. The treatment of ALS requires a comprehensive approach, involving neurologists, respiratory therapists, physical therapists, occupational therapists, speech therapists, nutritionists, and mental health professionals. Symptom management includes medications for muscle cramps, excess saliva, and emotional lability. As the disease progresses, assistive devices like wheelchairs, communication aids, and respiratory support become necessary. Nutritional support through a feeding tube may be recommended when swallowing difficulties develop.

• The use of enteral nutrition (EN) is recommended over parenteral nutrition (PN) in ALS patients who require medical nutrition therapy, as it is associated with fewer complications and better outcomes 1.
• Physical activity, including endurance and resistance exercises, can slow the progression of the disease and improve functionality and quality of life, and should be advised as long as it does not worsen the physical state of the patient 1.
• Early intervention with these treatments can improve quality of life and potentially extend survival time for ALS patients.
• The 2024 National Academies of Sciences, Engineering, and Medicine report emphasizes the importance of equitable access to affordable multidisciplinary care, services, and caregiver support, and recommends a reimagined, inclusive, and integrated multidisciplinary care and research system 1.
• The report also highlights the need for advances in basic science, clinical care, and population health research to improve survival and enhance quality of life for those living with or at-risk for ALS.

From the FDA Drug Label

Riluzole tablets is indicated for the treatment of amyotrophic lateral sclerosis (ALS) Riluzole tablets is indicated for the treatment of amyotrophic lateral sclerosis (ALS) Riluzole tablets is indicated for the treatment of amyotrophic lateral sclerosis (ALS) The treatment for ALS is Riluzole.

• The recommended dosage is 50 mg twice daily, taken at least 1 hour before or 2 hours after a meal 2.
• Riluzole may cause hepatotoxicity, neutropenia, and interstitial lung disease 2.
• Patients with a history of severe hypersensitivity reactions to Riluzole or any of its components should not use this medication 2.

From the Research

ALS Treatment Overview

• ALS (Amyotrophic Lateral Sclerosis) is a progressive neurodegenerative disease with limited treatment options.
• Riluzole is a commonly used medication for ALS, which has been shown to prolong survival by 2-3 months 3, 4.

Riluzole Efficacy and Safety

• Riluzole has been found to be reasonably safe and effective in prolonging median survival in patients with ALS 3, 4.
• The optimal time and duration of riluzole treatment are unknown, but early initiation may improve absolute survival 5.
• Riluzole has been found to have a small beneficial effect on bulbar and limb function, but not on muscle strength 3.

Symptomatic Treatments for ALS

• There is limited evidence for the effectiveness of symptomatic treatments for ALS, including pain management, cramp treatment, and spasticity management 6.
• Non-invasive ventilation (NIV) has been found to improve median survival and quality of life in people with respiratory insufficiency and normal to moderately impaired bulbar function 6, 7.
• Multidisciplinary team management is important for supporting people with ALS and their caregivers, and for improving outcomes 7.

Future Research Directions

• More research is needed to address the changing needs of people with ALS and their caregivers, and to assess the effectiveness of interventions for symptoms relating to ALS 6.
• Future studies should use appropriate outcome measures and have sufficient duration to address the progression of ALS and mortality 6.