First-Line Treatment for Bulbar ALS
The first-line treatment for bulbar ALS is riluzole 50 mg twice daily, combined with immediate multidisciplinary supportive care including nutritional assessment, swallowing evaluation, and early palliative care integration from the time of diagnosis. 1, 2
Disease-Modifying Pharmacotherapy
Riluzole 50 mg orally twice daily is the only FDA-approved disease-modifying medication for ALS and should be initiated immediately upon diagnosis. 2 Clinical trials demonstrate riluzole extends survival by approximately 60-90 days in the overall ALS population, with real-world evidence suggesting potentially greater benefit (up to 19 months median survival extension). 2, 3, 4 Importantly, riluzole appears particularly effective in bulbar-onset ALS patients, with one population-based study showing significant survival benefit specifically in this subgroup (p=0.04). 5
Critical Monitoring for Riluzole
- All adverse events from riluzole occur within the first 6 months of treatment, requiring vigilant monitoring during this period. 6
- Monitor liver enzymes before initiation and regularly during the first 6 months, as elevated transaminases are the most common reason for discontinuation (5.4% of patients). 6
- Obtain baseline chest X-ray and monitor for interstitial pneumonia, which can cause severe respiratory failure requiring steroid treatment. 6
- Assess for nausea, dizziness, general malaise, and asthenia at each visit during the first 6 months. 6, 4
Immediate Multidisciplinary Interventions
Swallowing Assessment and Nutritional Management
All patients with bulbar ALS require swallow screening before initiating any oral intake. 7 If screening is positive or the patient has high aspiration risk, proceed immediately to videofluoroscopy swallowing study (VFSS) or fiberoptic endoscopic evaluation of swallowing (FEES). 7
Conduct nutritional status assessment (BMI, weight loss) every 3 months to detect early malnutrition. 1 For patients with dysphagia:
- Adapt food texture to facilitate swallowing—use thicker liquids and semisolid foods with high water content instead of thin liquids. 8, 1
- Implement postural maneuvers, particularly chin-tuck posture, to protect the airway during swallowing. 8, 1
- Fractionate and enrich meals with energy-dense foods if muscular fatigue causes prolonged meal times. 8
- Initiate oral nutritional supplementation if weight loss progresses despite dietary modifications. 8
Plan for percutaneous endoscopic gastrostomy (PEG) placement early, ideally when FVC remains >50% of predicted, and refuse placement when FVC falls below 30%. 1 This timing is critical because bulbar-onset ALS has median survival of only 25-28 months from symptom onset. 1
Respiratory Management
Initiate non-invasive ventilation (NIV) for patients meeting any of these criteria: 1
- FVC <80% of normal with symptoms
- FVC <50% predicted
- Evidence of sleep-disordered breathing or hypoventilation on polysomnography
Use bilevel positive airway pressure (BPAP) with backup respiratory rate rather than standard CPAP, as this achieves better patient-ventilator synchrony in patients with bulbar impairment. 1 However, recognize that patients with significant bulbar dysfunction may not tolerate NIV or achieve adequate ventilation. 7
Sialorrhea Management
First-line therapy for sialorrhea is an inexpensive oral anticholinergic medication; second-line therapy is botulinum toxin injection to salivary glands. 1 Monitor for dry mouth as a potential side effect of anticholinergic medications. 7
Palliative Care Integration
Adopt a palliative care approach from the time of diagnosis, not reserved for end-stage disease, with early referral to palliative services. 1 This is essential because speech and communication become severely limited in later stages, making advance care planning discussions impossible if delayed. 1
Initiate end-of-life discussions at specific trigger points: 1
- Presence of patient distress
- Evolution of disease requiring major interventions (PEG, NIV)
- Expressed desire of the patient to discuss these issues
Critical Pitfalls to Avoid
Equipment and service delays can result in catastrophic safety risks—all requests for equipment and services for bulbar ALS should be considered urgent and handled expeditiously. 1 Late palliative care referral negatively impacts patient outcomes. 1
Aspiration pneumonia occurs in 11.4-13% of bulbar ALS cases and represents a leading cause of mortality. 1 Weight loss and malnutrition develop in 0-21% of patients at diagnosis and worsen progressively, both of which are detrimental to survival and quality of life. 1, 7
Bulbar-onset patients demonstrate significantly worse outcomes compared to spinal-onset, with median survival of 28 months versus 44 months for spinal-onset patients with dysphagia. 1 This compressed timeline necessitates aggressive early intervention.
Multidisciplinary Team Composition
The care team should include neurology, pulmonology, gastroenterology, speech-language pathology, nutrition, physical therapy, occupational therapy, social work, and palliative care. 1 Multidisciplinary care improves both survival and quality of life in ALS. 1