Management Approach for Progressive Supranuclear Palsy (PSP)
The management of Progressive Supranuclear Palsy requires a multidisciplinary team approach focusing on symptomatic treatment, as there are currently no disease-modifying therapies available.
Core Management Principles
- PSP is a relentlessly progressive neurodegenerative condition requiring a palliative approach from diagnosis with emphasis on patient autonomy, dignity, and quality of life 1
- Early palliative care involvement is essential as it improves quality of life in patients and caregivers 1
- Management should be led by a multidisciplinary team including neurologists, physiotherapists, occupational therapists, speech and language therapists, dieticians, ophthalmologists, psychologists, and palliative care specialists 2
Pharmacological Management
- Levodopa may provide limited symptomatic relief for parkinsonian features (bradykinesia and rigidity), though benefits are often negligible and short-lived 2
- Intrasalivary gland botulinum toxin is useful for managing problematic sialorrhea 2
- Intramuscular botulinum toxin and baclofen can help reduce dystonia, including blepharospasm 2
- Benzodiazepines may be useful in managing dystonia but should be used cautiously due to risk of falls 2
- Levetiracetam and benzodiazepines can be considered for myoclonus 2
- Acetylcholinesterase inhibitors and NMDA receptor antagonists have limited evidence of efficacy and may cause adverse effects that outweigh benefits 2
- Antidepressants may help with behavioral symptoms and depression but are often poorly tolerated 2
Non-Pharmacological Interventions
- Physiotherapy rehabilitation programs targeting gait, balance, and physical capability show preliminary evidence of effectiveness 3
- Specific interventions for gaze control should be included in rehabilitation programs 3
- Regular monitoring and appropriate adjustment of therapy is necessary as the disease progresses 4
- Assistive devices are required by approximately 90% of patients during their disease course 4
- Supportive care services should be implemented early and utilized by most patients (86%) 4
Management of Neuropsychiatric Symptoms
- A structured approach like DICE (Describe, Investigate, Create, Evaluate) should be used to manage behavioral symptoms 5
- Non-pharmacological interventions should be considered first, including structured individualized activities, predictable daily routines, and improved communication techniques 5
- Investigate potential underlying causes including medication side effects, undetected medical conditions, pain, or functional limitations 5
Disease Progression and Monitoring
- Early symptoms often include falls (median 2.0 years before diagnosis), unsteady gait (median 1.2 years before diagnosis), and mobility problems (median 0.8 years before diagnosis) 4
- Regular monitoring of disease progression is essential for adjusting management strategies 4
- Imaging studies are utilized by 99% of patients during their disease course and can help monitor progression 4
Common Pitfalls and Caveats
- Delayed diagnosis is common, leading to inappropriate treatment and increased morbidity 6
- Atypical antipsychotics for behavioral symptoms should be avoided as they can worsen parkinsonism 2
- Falls are a major source of morbidity and should be anticipated with early implementation of fall prevention strategies 6
- Aspiration pneumonia is the leading cause of death, necessitating early swallowing assessment and management 3
- Excessive focus on pharmacological management may detract from the benefits of non-pharmacological approaches 2
End-of-Life Care
- Advance care planning should be initiated early in the disease course 1
- Palliative care services should be integrated throughout the disease trajectory rather than only at end-of-life 1
- Management of symptoms at end-of-life requires careful consideration of medication side effects and quality of life 1