What is the primary treatment for amyotrophic lateral sclerosis (ALS)?

Primary Treatment for Amyotrophic Lateral Sclerosis (ALS)

Riluzole (50 mg twice daily) is the primary disease-modifying treatment for amyotrophic lateral sclerosis (ALS), as it is the only FDA-approved medication shown to extend survival in ALS patients. 1

Disease-Modifying Therapy

• 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
• Clinical trials have demonstrated that riluzole extends survival by approximately 2-3 months in ALS patients 2, 3
• The medication works by inhibiting glutamate release, which helps reduce excitotoxicity, a key pathogenic mechanism in ALS 4

Monitoring and Safety Considerations

• Serum aminotransferases should be measured before and during treatment with riluzole 1
• Riluzole is contraindicated in patients with a history of severe hypersensitivity reactions to the drug and is not recommended in patients with baseline elevations of serum aminotransferases greater than 5 times the upper limit of normal 1
• The most common adverse effects include:
  • Asthenia (weakness) - reported in approximately 18% of patients 5
  • Nausea - reported in approximately 15% of patients 5
  • Dizziness, decreased lung function, and abdominal pain 1
• Liver enzyme monitoring is crucial as approximately 10-15% of patients may experience alanine aminotransferase elevations more than three times the upper limit of normal 5
• Patients should be monitored for signs of neutropenia and advised to report any febrile illness 1

Supportive Care Measures

• Nutritional management is essential as ALS progresses:

  • Regular nutritional status assessment (BMI, weight loss) every 3 months 6
  • For patients with dysphagia, food texture should be adapted to facilitate swallowing 6, 7
  • Enteral nutrition via feeding tubes (preferably gastrostomy) is recommended when oral intake becomes insufficient 6, 7

• Physical activity recommendations:

  • Low to moderate physical activity should be advised as long as it doesn't worsen the patient's condition 7
  • Excessive physical exertion that could lead to fatigue should be avoided 7

Palliative Care

• A palliative care approach should be adopted from the time of diagnosis, with early referral to palliative services 6
• This helps establish relationships with staff and address end-of-life issues proactively 6

Common Pitfalls and Caveats

• Riluzole should be discontinued if there is evidence of liver dysfunction or if interstitial lung disease develops 1
• Drug interactions are important to consider:
  • Strong to moderate CYP1A2 inhibitors may increase riluzole-associated adverse reactions 1
  • Strong to moderate CYP1A2 inducers may result in decreased efficacy 1
  • Concomitant use with other hepatotoxic drugs may increase risk for hepatotoxicity 1
• Despite its proven efficacy, riluzole provides only modest survival benefits, highlighting the need for comprehensive supportive care 3
• 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