What is the management approach for progressive supranuclear palsy (PSP)?

Management of Progressive Supranuclear Palsy (PSP)

PSP requires multidisciplinary supportive care focused on symptomatic management, as no disease-modifying therapies currently exist, with treatment centered on addressing motor symptoms, dysphagia, nutritional status, and behavioral complications through both pharmacological and non-pharmacological interventions. 1, 2

Diagnostic Confirmation and Clinical Recognition

PSP classically presents in the sixth or seventh decade (mean age 63) with postural instability, unexplained falls, axial dystonia, and vertical supranuclear gaze palsy (though gaze palsy typically appears later in disease course). 3 Early symptoms include:

• Lurching gait and axial dystonia manifested as unexplained falls 3
• Ocular symptoms including blurred vision and slow saccades early, with vertical supranuclear gaze palsy developing later 3
• Bradykinesia and rigidity with additional clinical features beyond typical Parkinson's disease 3

Neuroimaging Findings

MRI demonstrates characteristic patterns of regional volume loss, with FDG-PET showing hypometabolism in medial frontal and anterior cingulate cortices, striatum, and midbrain—findings that distinguish PSP from idiopathic Parkinson's disease. 3

Pharmacological Management

Motor Symptoms

While no medications can reverse disease progression, limited symptomatic benefit may be attempted with dopaminergic agents, though response is typically poor compared to Parkinson's disease. 1, 2

Cognitive and Behavioral Symptoms

For cognitive-behavioral disturbances, consider trial of cholinesterase inhibitors (rivastigmine) combined with antidepressants (SSRIs), as case reports demonstrate clinically significant improvement in cognition and function with this combination. 4

• Start SSRIs at low doses (citalopram 10 mg/day or sertraline 25-50 mg/day) for behavioral symptoms including apathy, depression, and irritability 4
• Rivastigmine may provide benefit for cognitive symptoms when combined with antidepressant therapy 4

Agitation and Aggression Management

When behavioral symptoms become severe:

First-line approach: Systematically investigate and treat reversible causes including pain, urinary tract infections, constipation, dehydration, and medication side effects before considering psychotropic medications. 5

Second-line pharmacological treatment (only after behavioral interventions fail):

• For chronic agitation without psychotic features: SSRIs (citalopram 10-40 mg/day or sertraline 25-200 mg/day) 5
• For severe agitation with psychotic features: Risperidone 0.25-1.25 mg/day, starting at 0.25 mg at bedtime 5
• Evaluate response within 4 weeks using quantitative measures; taper and discontinue if no benefit 5

Critical safety warning: All antipsychotics increase mortality risk (1.6-1.7 times higher than placebo) in elderly patients with neurodegenerative disease; discuss risks with patient/surrogate before initiating. 5

Nutritional and Dysphagia Management

Regular monitoring of nutritional and vitamin status is essential throughout disease progression, with particular attention to weight changes and supplementation needs (vitamin D, folate, vitamin B12). 6

Weight loss is common and associated with disease progression, partly due to increased energy expenditure from rigidity. 6

Dysphagia Protocol

Evaluate and manage dysphagia through:

• Adaptation of food bolus characteristics (texture modification) 6
• Postural maneuvers during swallowing 6
• Individualized exercise programs following multidimensional swallowing assessment 6

Non-Pharmacological Interventions

Fall Prevention and Mobility

Given that falls occur early (median 2.0 years before diagnosis) and mobility problems develop rapidly (median 0.8 years before diagnosis), immediate implementation of fall prevention strategies is critical. 7

• Assistive devices are utilized by 90% of patients at some point during disease course 7
• Physical therapy consultation for gait training and transfer techniques 1
• Home safety modifications including removal of hazardous items and installation of handrails 5

Supportive Care Resources

At least 86% of PSP patients utilize supportive care services during disease progression, making early referral to multidisciplinary services essential. 7

Telehealth Nursing Intervention

Telephonic nursing case management (Cure PSP Care Guide model) provides knowledge, guidance, and resource identification to patients and caregivers, improving caregiver knowledge and addressing daily management needs. 8

Monitoring and Disease Trajectory

The mean disease duration is approximately 6 years from diagnosis, with rapid progression requiring frequent reassessment. 3

Key monitoring parameters:

• Nutritional status and weight at every visit 6
• Swallowing function and aspiration risk 6
• Fall frequency and mobility decline 7
• Cognitive and behavioral symptoms using quantitative measures 5
• Medication side effects, particularly extrapyramidal symptoms and metabolic changes 5

Critical Pitfalls to Avoid

• Do not expect significant response to dopaminergic therapy as seen in Parkinson's disease; PSP demonstrates poor levodopa responsiveness 3, 1
• Do not use typical antipsychotics (haloperidol) as first-line for behavioral symptoms due to 50% risk of tardive dyskinesia after 2 years in elderly patients 5
• Do not use benzodiazepines routinely for agitation due to increased delirium risk, cognitive impairment, and paradoxical agitation in 10% of elderly patients 5
• Do not continue antipsychotics indefinitely without regular reassessment at every visit 5
• Do not overlook pain as a major contributor to behavioral disturbances in patients who cannot verbally communicate discomfort 5