What is the best management approach for a patient over 50 with Motor Neurone Disease (MND) and potential pre-existing medical conditions?

Management of Motor Neurone Disease (MND) in Patients Over 50

Multidisciplinary specialist care is the cornerstone of MND management and has proven survival benefits, with non-invasive ventilation (NIV) being the single most important intervention that extends median survival by approximately 4.5 months while maintaining quality of life. 1

Core Management Framework

Multidisciplinary Team Approach

• Establish care with a specialized MND multidisciplinary team immediately upon diagnosis, as this framework has demonstrated both survival benefits and improved quality of life outcomes 2, 3, 4
• The team must include a metabolic/neurological physician, pulmonologist, gastroenterologist, physiotherapist, occupational therapist, speech therapist, and palliative care specialist 5, 4
• General practitioners serve as critical coordinators between hospital-based specialty care and community services 5

Respiratory Management (Priority #1 for Survival)

Non-Invasive Ventilation Implementation

• Initiate NIV consideration in ANY breathless or acutely unwell MND patient BEFORE respiratory acidosis develops, as any elevation of pCO2 signals impending crisis 1
• NIV significantly improves survival: median 15.41 months with NIV versus 10.88 months without 1
• Start with low pressure support (IPAP 10-20 cm H2O) in patients without chest wall distortion, using an I:E ratio of 1:1 1

Critical Warning Signs Requiring Immediate Escalation

• Bulbar dysfunction combined with profound hypoxemia or rapid desaturation during NIV breaks mandates HDU/ICU placement, as these patients have higher NIV failure rates 1
• Deterioration can be sudden due to reduced respiratory reserve, impaired cough, and potentially undiagnosed cardiomyopathy 1
• Recovery from acute respiratory failure takes longer than COPD, requiring slower NIV weaning with target pCO2 around 6.5 kPa 1

Invasive Mechanical Ventilation Considerations

• Avoid emergent invasive mechanical ventilation when possible, as outcomes are poor: median survival only 250 days when initiated emergently, with only 17% successfully weaned 1
• Discuss with specialist centers before proceeding with IMV given complexity and limited outcomes 1
• Elective intubation rates vary significantly by region (0.8-10.6%), reflecting different practice philosophies rather than evidence 1

Nutritional Management

Dysphagia Interventions

• Implement chin-tuck posture as the primary protective maneuver for the majority of MND patients with dysphagia, as it opens the valleculae and prevents laryngeal penetration 1
• Use modified-consistency foods and thickened liquids as alternatives to thin liquids to reduce aspiration risk 1
• Instruct patients to throat clear every 3-4 swallowing acts to prevent postswallowing inhalation, particularly in the 23% with penetration without aspiration 1

Nutritional Supplementation Strategy

• Prioritize caloric supplementation with carbohydrates over high-fat supplementation, as carbohydrate-based approaches show better survival outcomes 1
• Weight maintenance is critical—weight loss correlates with more rapid disease progression 2, 3
• Provide nutritional supplementation when enriched diet alone fails to meet requirements 1

Physical Therapy and Exercise

• Use submaximal effort strengthening regimens designed to prevent disuse atrophy while avoiding exercise-induced muscle injury 1
• Avoid high-intensity strengthening exercises due to concern about precipitating muscle breakdown in progressive MND 1
• Specialized physiotherapy is essential for sputum clearance, as bulbar dysfunction renders voluntary cough less effective 1

Cardiac Considerations in Older Patients

Arrhythmia Management

• Consider thrombosis prophylaxis in MND patients with normal systolic function and atrial fibrillation/flutter, with therapy type determined by individual thrombosis risk 1
• Use antiarrhythmic drugs (class I, II, or IV) cautiously, as they can increase peripheral muscular weakness; tailor treatment to coexisting conduction abnormalities 1

Cardioverter-Defibrillator Therapy

• Apply standard criteria for primary ICD therapy: class II or III heart failure with LVEF ≤35% despite medical therapy 1

Monitoring and Follow-Up Protocol

Regular Assessments Required

• Baseline and periodic ophthalmologic examination with dilated funduscopy 6
• Serial chest radiographs and pulmonary function tests to assess lung involvement 6
• Regular assessment of growth and nutritional status 6
• Continuous monitoring for hepatosplenomegaly and hypersplenism (though this is more relevant for Niemann-Pick disease, not MND) 6

Specialized Physiotherapy

• Essential for aiding sputum clearance given impaired voluntary cough from bulbar dysfunction 1

Palliative Care Integration

• Integrate palliative care early in disease management, not just at end-of-life 2, 3
• Discuss end-of-life care planning and advance directives early with patients and families 6
• The end-of-life phase is poorly defined in MND, but effective symptom control through palliative care is achievable and essential 2, 3
• Most patients die from pulmonary infections or respiratory failure; death by choking is rare, and final stages are usually peaceful and dignified 7

Common Pitfalls to Avoid

• Do not deny acute NIV to MND patients presenting with acute hypercapnic respiratory failure—these individuals can survive long-term on home NIV with good quality of life even after severe initial presentation 1
• Do not wait for respiratory acidosis before considering NIV—intervene at first signs of breathlessness 1
• Do not assume all MND patients are inappropriate for aggressive respiratory support—outcomes with planned NIV are significantly better than crisis management 8
• Avoid high-intensity exercise programs that may accelerate muscle breakdown 1