What is the recommended treatment plan for a patient newly diagnosed with Amyotrophic Lateral Sclerosis (ALS)?

Treatment Plan for Newly Diagnosed ALS

For a patient newly diagnosed with ALS, immediately initiate riluzole 50 mg twice daily (taken at least 1 hour before or 2 hours after meals) and establish multidisciplinary care with integrated palliative services from the time of diagnosis. 1, 2

Disease-Modifying Pharmacotherapy

First-Line Therapy

• Start riluzole 50 mg orally twice daily on an empty stomach (at least 1 hour before or 2 hours after meals), which reduces risk of death by 35% and extends median survival by 2-19 months depending on the population studied 1, 3, 4, 5
• Measure serum aminotransferases (liver enzymes) before starting riluzole and monitor during treatment; discontinue if levels exceed 5 times upper limit of normal 1
• Advise patients to report any febrile illness due to risk of neutropenia 1

Additional Disease-Modifying Options

• Consider adding edaravone 60 mg IV infusion over 60 minutes using the following schedule: initial cycle of daily dosing for 14 days followed by 14-day drug-free period, then subsequent cycles of daily dosing for 10 days out of 14-day periods followed by 14-day drug-free periods 6
• Edaravone has demonstrated statistically significant slowing of ALS disease progression in clinical trials 7
• Screen for history of hypersensitivity reactions and sulfite allergies before initiating edaravone 6

Nutritional Management

Initial Assessment and Monitoring

• Perform nutritional screening at diagnosis measuring BMI and weight loss, then repeat every 3 months to detect early malnutrition 8, 2
• Conduct videofluoroscopy swallowing study at diagnosis even in asymptomatic patients to detect early dysphagia, as aspiration can occur without clinical signs 8, 9, 10

Nutritional Goals Based on BMI

• If BMI <25 kg/m²: recommend weight gain 8
• If BMI 25-35 kg/m²: recommend weight stabilization 8
• If BMI >35 kg/m²: recommend weight loss to improve passive and active mobilization 8
• Weight loss >5-10% of habitual weight is associated with decreased survival (RR 4.18 for >10% weight loss) 8

Energy Requirements

• Estimate energy needs at approximately 30 kcal/kg body weight in non-ventilated patients, adjusted for physical activity level and body composition changes 8
• Indirect calorimetry is preferred when available, as predictive equations have limits of agreement ranging from -677 to +591 kcal/day 8

Dysphagia Management Strategies

• Modify food texture to ease oral and pharyngeal transport while preventing choking 2
• Implement chin-tuck posture during swallowing to prevent laryngeal penetration 2
• Use thicker liquids and semisolid foods with high water content instead of thin liquids 2, 9
• Recommend eating several small meals throughout the day with meal enrichment using high-calorie foods 2
• Add dietary fiber if constipation develops from abdominal weakness 2

Enteral Nutrition Timing

• Place percutaneous endoscopic gastrostomy (PEG) when forced vital capacity (FVC) remains >50% predicted and before it falls below 30% 10
• Gastrostomy is preferable to parenteral nutrition for long-term nutritional support 8
• Weight loss >10% at time of PEG placement is associated with increased mortality 8

Respiratory Management

Baseline Assessment

• Establish baseline pulmonary function with slow vital capacity (SVC) measurements and peak cough flow (PCF) to assess airway clearance ability 9

Non-Invasive Ventilation Criteria

• Initiate NIV when any of the following occur: 10
  • FVC <80% of normal with symptoms of respiratory insufficiency
  • FVC <50% predicted
  • Evidence of sleep-disordered breathing or hypoventilation on polysomnography
• Use bilevel positive airway pressure (BPAP) with backup respiratory rate for better patient-ventilator synchrony, especially in patients with bulbar impairment 10

Important Caveat

• Screen for cognitive impairment before recommending NIV, as up to 40% of ALS patients have cognitive dysfunction that reduces NIV compliance 2, 10
• Patients with executive dysfunction are at higher risk of falls, choking episodes, and injuries 10

Symptomatic Management

Sialorrhea (Excessive Salivation)

• First-line: oral anticholinergic medication (inexpensive option) 10
• Second-line: botulinum toxin A injections to salivary glands if anticholinergics ineffective 2, 10

Physical Activity

• Recommend endurance and resistance exercises as long as they do not worsen physical state, as they may slow disease progression and improve quality of life 8

Palliative Care Integration

Timing and Approach

• Adopt palliative care approach from the time of diagnosis, not reserved for end-stage disease 2, 9, 10
• Refer to palliative services immediately to establish relationships with staff before speech and communication become severely limited 2, 9, 10
• Multidisciplinary care improves both survival and quality of life in ALS 2, 10

Advance Care Planning

• Initiate advance directive discussions at diagnosis, including preferences regarding ventilatory support, feeding tubes, and end-of-life care 2, 10
• Trigger points for end-of-life discussions include patient distress, disease evolution, or patient's expressed desire to discuss these issues 10
• Only 30% of patients complete advance directives despite 78% of centers considering them useful 2

Multidisciplinary Team Composition

• Assemble team including: neurology, pulmonology, gastroenterology, speech-language pathology, nutrition, physical therapy, occupational therapy, social work, and palliative care 10

Caregiver Support

• Implement structured caregiver support from diagnosis, including counseling, support groups, and crisis management systems 2, 10
• Behavioral deficits in ALS patients have significant negative impact on caregivers' quality of life 2

Critical Pitfalls to Avoid

• Never delay equipment or service requests - all requests for ALS patients should be considered urgent and handled expeditiously, as delays can result in catastrophic safety risks rather than simple inconvenience 8, 10
• Never delay palliative care referral - late referral is the most common and harmful error, negatively impacting quality of life for both patients and caregivers 2, 10
• Never wait for symptomatic dysphagia to perform swallowing evaluation - videofluoroscopy should be done at diagnosis as aspiration can be silent 8, 9
• Never delay gastrostomy placement until respiratory function severely deteriorates - PEG should be placed before FVC falls below 50% predicted 10