From the Guidelines
For patients with progressive supranuclear palsy (PSP), pulmonary management should focus on preventing aspiration pneumonia and respiratory complications, with a strong emphasis on individualized non-invasive positive pressure ventilation (NPPV) titration to address sleep-disordered breathing, as outlined in the best clinical practices for NPPV adjustment in stable chronic alveolar hypoventilation syndromes 1. Pulmonary care for PSP patients involves several key strategies:
- Regular swallowing assessments to identify aspiration risk, with modifications to food textures as needed, and consideration for percutaneous endoscopic gastrostomy (PEG) tube placement in advanced cases.
- Chest physiotherapy with postural drainage and assisted coughing techniques to improve secretion clearance, which is crucial given the progressive bulbar dysfunction and impaired cough reflexes associated with PSP.
- Utilization of mechanical insufflation-exsufflation devices, such as CoughAssist, when cough effectiveness diminishes, to aid in effective airway clearance.
- Prophylactic vaccinations against pneumococcal pneumonia and annual influenza to prevent respiratory infections, which can be particularly severe in PSP patients due to their compromised respiratory function.
- Prompt antibiotic therapy for acute respiratory infections, typically starting with amoxicillin-clavulanate 875/125 mg twice daily for 7-10 days or appropriate alternatives based on local resistance patterns, to minimize the risk of complications. In the context of NPPV for PSP patients, careful mask fitting and a period of acclimatization to low pressure prior to titration, as well as individualized treatment goals, are essential for effective nocturnal ventilatory support 1. These goals typically include preventing worsening of hypoventilation during sleep, improving sleep quality, relieving nocturnal dyspnea, and providing respiratory muscle rest, all of which are critical for maintaining quality of life and reducing morbidity and mortality in PSP patients.
From the Research
Pulmonary Management for Progressive Supranuclear Palsy
From a pulmonary standpoint, managing a patient with progressive supranuclear palsy (PSP) involves addressing the respiratory complications associated with the disease. Key considerations include:
- Dysphagia and aspiration pneumonia, which are common in PSP patients 2, 3
- Cough dysfunction, which can be improved with sensorimotor training in airway protection (smTAP) 2
- The need for a multidisciplinary approach to care, including patient-centered and symptomatic treatment strategies 4, 5
Treatment Strategies
Current treatment strategies for PSP are primarily symptomatic and supportive, with a focus on managing the disease's various symptoms, including:
- Respiratory symptoms, such as dysphagia and cough dysfunction 2
- Motor symptoms, such as falls and gaze palsy 4, 5
- Non-motor symptoms, such as cognitive impairment and behavioral changes 4, 5
Future Directions
Ongoing research is focused on developing disease-modifying therapies, including tau-based therapeutics, which may offer new treatment options for PSP patients in the future 5, 3. Additionally, studies on the feasibility of cough rehabilitation in PSP, such as the use of smTAP, may provide new insights into managing respiratory complications in these patients 2.