What is the management approach for Progressive Supranuclear Palsy (PSP)?

Management of Progressive Supranuclear Palsy (PSP)

There is no disease-modifying treatment for PSP; management must focus on multidisciplinary symptomatic relief and optimizing quality of life through targeted interventions for specific symptoms, with physiotherapy for balance and gait being the most evidence-supported intervention. 1, 2

Core Management Principles

Progressive supranuclear palsy requires a fundamentally different approach than other parkinsonian disorders because:

• Levodopa provides minimal to no benefit in PSP and "poor levodopa response" is actually part of the diagnostic criteria, making it inappropriate as a primary treatment strategy 1
• The focus must be entirely symptomatic and supportive, as no pharmacological agents modify disease progression 1, 2
• Multidisciplinary team management is essential and should include neurologists, physiotherapists, occupational therapists, speech and language therapists, dieticians, ophthalmologists, psychologists, and palliative care specialists 1

Symptom-Specific Treatment Algorithm

1. Mobility, Balance, and Falls (Primary Concern)

Physiotherapy rehabilitation programs targeting gait, balance, and gaze control are the most evidence-supported intervention for PSP:

• Multiple studies demonstrate improvement in balance, gait impairment, and reduction of falls after physiotherapy interventions 3, 4
• Treadmill training and robot-assisted gait training show particular promise in improving mobility outcomes 4
• Physical exercises should be initiated early and maintained throughout disease course 3, 4

Common pitfall: Delaying physiotherapy referral until advanced disease stages reduces effectiveness; early intervention is critical 3

2. Parkinsonian Symptoms (Rigidity and Bradykinesia)

A trial of levodopa may be attempted but expectations must be realistic:

• Levodopa may provide minimal, short-lived improvement in rigidity and bradykinesia in some patients 1
• Evidence is conflicting and benefits are often negligible 1
• If no response after adequate trial (typically 1000-1500 mg/day for several weeks), discontinue to avoid unnecessary side effects 1

Critical warning: Do not persist with levodopa if there is no clear benefit, as side effects may worsen quality of life without therapeutic gain 1

3. Dystonia and Blepharospasm

Botulinum toxin injections are the most effective treatment:

• Intramuscular botulinum toxin for limb dystonia and blepharospasm shows clear benefit 1
• Baclofen (oral or intrathecal) can be used as adjunctive therapy for dystonia 1
• Benzodiazepines may provide additional benefit for dystonia management 1

4. Sialorrhea (Drooling)

Intrasalivary gland botulinum toxin injections are effective:

• Evidence supports botulinum toxin for managing problematic sialorrhea in PSP 1
• This intervention significantly improves quality of life and reduces aspiration risk 1

5. Myoclonus

Levetiracetam is the preferred agent:

• Levetiracetam is effective for myoclonus management 1
• Benzodiazepines can be used as alternative or adjunctive therapy 1

6. Cognitive and Behavioral Symptoms

Avoid acetylcholinesterase inhibitors and memantine:

• These agents (donepezil, rivastigmine, galantamine, memantine) have limited evidence of effectiveness in PSP 1
• Risk of adverse effects may outweigh any potential benefits 1

For depression and behavioral symptoms:

• Antidepressants (SSRIs) may be useful but are often poorly tolerated due to adverse effects 1
• Monitor closely for tolerability and discontinue if no clear benefit 1

Critical warning: Avoid antipsychotics in PSP as they worsen parkinsonism and carry increased mortality risk in elderly patients with neurodegenerative conditions 1

7. Speech and Swallowing Dysfunction

Early speech and language therapy referral is essential:

• Swallowing problems are common and aspiration pneumonia is the leading cause of death in PSP 3
• Speech therapy should address both communication difficulties and dysphagia 3
• Despite the importance, there is insufficient research on specific speech therapy interventions for PSP 3

Common pitfall: Delaying swallowing assessment until aspiration events occur; proactive evaluation and management reduces pneumonia risk 3

8. Visual Symptoms (Supranuclear Gaze Palsy)

Ophthalmology consultation and adaptive strategies:

• Prism glasses may help with downgaze palsy 2
• Physiotherapy programs incorporating gaze control training show benefit 3, 4
• Environmental modifications to reduce fall risk related to visual impairment 2

What NOT to Do

Avoid these common management errors:

• Do not use typical or atypical antipsychotics for behavioral symptoms as they worsen parkinsonism and increase mortality risk 1
• Do not continue levodopa indefinitely without clear documented benefit 1
• Do not delay multidisciplinary team involvement until advanced disease stages 1, 2
• Do not pursue deep brain stimulation as surgical approaches have been ineffective in PSP 5

Palliative Care Integration

Early palliative care involvement improves quality of life:

• Palliative care should be integrated from diagnosis, not reserved for end-stage disease 6
• Focus on patient autonomy, dignity, and quality of life throughout disease course 6
• Address caregiver burden proactively as it significantly impacts outcomes 6

Monitoring and Follow-Up

Use standardized measures to track progression and treatment response:

• Progressive Supranuclear Palsy Rating Scale (PSPRS) is the most commonly used outcome measure 4
• Unified Parkinson's Disease Rating Scale (UPDRS) can also be utilized 4
• Regular reassessment of fall risk, swallowing function, and mood is essential 2