What is the primary treatment for Amyotrophic Lateral Sclerosis (ALS)?

Primary Treatment for Amyotrophic Lateral Sclerosis (ALS)

Riluzole is the primary disease-modifying treatment for ALS, with edaravone as a more recently approved option to slow disease progression. 1, 2

Disease-Modifying Medications

• Riluzole is indicated for the treatment of ALS with a recommended dosage of 50 mg twice daily, taken at least 1 hour before or 2 hours after meals 1
• Riluzole has been demonstrated to extend survival in ALS patients, decreasing the risk of death or tracheostomy by 35% compared to placebo 3
• Edaravone (Radicava) was approved by the FDA in 2017 as the second disease-modifying drug for ALS, administered as a 60 mg intravenous infusion in 28-day cycles 4
• Edaravone has been shown to slow the loss of physical function in ALS patients by 33% compared to placebo 4

Monitoring and Safety Considerations

• For patients on riluzole, serum aminotransferases should be measured before and during treatment due to potential hepatic injury 1
• Riluzole is contraindicated in patients with baseline elevations of serum aminotransferases greater than 5 times the upper limit of normal 1
• The most common adverse reactions to riluzole (incidence ≥5%) include asthenia, nausea, dizziness, decreased lung function, and abdominal pain 1, 5
• Monitor for neutropenia in patients taking riluzole, as there is a suspicion it may cause this condition in rare cases 5

Multidisciplinary Care Approach

• Multidisciplinary care is essential for managing ALS and has been shown to prolong life and improve quality of life 2
• Regular nutritional status assessment every 3 months is recommended to detect early malnutrition 6
• For patients with dysphagia:
  • Adapt food texture to facilitate swallowing 7
  • Implement postural maneuvers, particularly chin-tuck posture, to protect the airway during swallowing 7
  • Use thicker liquids and semisolid foods with high water content instead of thin liquids 7, 6

Advanced Nutritional Support

• Enteral nutrition via feeding tubes (preferably gastrostomy) is recommended when oral intake becomes insufficient 6
• Percutaneous Endoscopic Gastrostomy (PEG) placement should be considered before respiratory function significantly deteriorates 6
• Parenteral nutrition should only be considered in acute settings when enteral nutrition is contraindicated or not feasible 7

Physical Activity Recommendations

• Low to moderate physical activity should be advised as long as it doesn't worsen the patient's condition 7
• Some evidence suggests that endurance and resistance exercises may slow disease progression and improve functionality and quality of life 7
• Avoid excessive physical exertion that could lead to fatigue and worsen symptoms 7

Palliative Care

• A palliative care approach should be adopted from the time of diagnosis 6
• Early referral to palliative services is recommended to establish relationships with staff and address end-of-life issues 6

Pitfalls and Caveats

• Despite numerous clinical trials, only riluzole and edaravone have demonstrated efficacy in slowing ALS progression 8, 2
• The rapidly progressive nature of ALS requires expedited approval and delivery of equipment and services to prevent catastrophic safety risks 9
• Dysphagia management techniques that work for other conditions may not be effective in ALS due to the specific pathophysiology of muscle atrophy and fatigue 7
• Despite limited evidence for efficacy of some interventions, nutritional support remains crucial for maintaining quality of life and preventing complications 7