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 FDA approval for slowing disease progression and extending survival. 1

Disease-Modifying Medications

• Riluzole is administered at a recommended dosage of 50 mg twice daily, taken at least 1 hour before or 2 hours after meals 1
• Riluzole works as an antiglutamatergic agent that has been shown to extend survival in ALS patients, though the effect is modest 2, 3
• Edaravone (Radicava) is another FDA-approved medication (since 2017) that can slow down the progression of ALS by approximately 33% compared to placebo by acting as a potent antioxidant and free radical scavenger 4, 5

Monitoring and Safety Considerations

• Regular monitoring of serum aminotransferases (liver enzymes) is essential before and during riluzole treatment 1
• Riluzole is contraindicated in patients with baseline elevations of serum aminotransferases greater than 5 times the upper limit of normal 1
• Common adverse effects of riluzole include asthenia (18%), nausea (15%), dizziness, decreased lung function, and abdominal pain 1, 2
• Vigilance for potential neutropenia is recommended in patients taking riluzole 2

Multidisciplinary Care Approach

• Multidisciplinary care is crucial for managing ALS symptoms and improving quality of life 5
• Regular nutritional status assessment (BMI, weight loss) should be performed every 3 months to detect early malnutrition 6
• For patients with dysphagia:
  • Adapt food texture to facilitate swallowing 7, 6
  • Implement postural maneuvers, particularly chin-tuck posture 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 7, 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
• 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 helps establish relationships with staff and address end-of-life issues 6

Pitfalls and Caveats

• Despite numerous clinical trials, only riluzole and edaravone have demonstrated significant benefits in ALS treatment 8, 5
• The rapidly progressive nature of ALS (average life expectancy of 2-5 years from onset) requires expedited approval and delivery of equipment and services 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