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Progressive Supranuclear Palsy (PSP)

(Steele-Richardson-Olszewski Syndrome)

By

Alex Rajput

, MD, University of Saskatchewan;


Eric Noyes

, MD, University of Saskatchewan

Reviewed/Revised Feb 2024
View PATIENT EDUCATION
Topic Resources

Progressive supranuclear palsy is a rare, degenerative central nervous system disorder that progressively impairs voluntary eye movements and causes bradykinesia, muscular rigidity with progressive axial dystonia, pseudobulbar palsy, and dementia. Diagnosis is clinical. Treatment focuses on relieving symptoms.

The cause of progressive supranuclear palsy is unknown.

Neurons in the basal ganglia and brain stem degenerate; neurofibrillary tangles containing an abnormally phosphorylated tau protein are also present.

Symptoms and Signs of Progressive Supranuclear Palsy

Symptoms of progressive supranuclear palsy usually begin in late middle age.

The first symptom may be

  • Difficulty looking up or down without moving the neck or difficulty climbing up and down stairs

Voluntary eye movements, particularly downward gaze, become difficult, but vertical reflex eye movements triggered by passive head movement (neck flexion, extension) are unaffected.

Movements are slowed, muscles become rigid, and axial dystonia develops. Patients tend to fall backward.

Dysphagia, dysarthria with emotional lability (pseudobulbar palsy), depression, and disordered sleep are common. Resting tremor may develop.

  • PSP with Richardson syndrome (PSP-RS): Classic progressive supranuclear palsy with progressive supranuclear ophthalmoplegia and severe balance problems (the most common form, in ≥ 70% of cases)

  • PSP with predominant Parkinsonism (PSP-P): Parkinsonian type of progressive supranuclear palsy, which responds to levodopa, resulting in transient, slight improvement

  • PSP with ocular motor dysfunction (PSP-OM): Characterized by oculomotor abnormalities (eg, vertical supranuclear gaze palsy)

  • PSP with progressive gait freezing (PSP-PGF): Characterized by progressive gait freezing

  • PSP with predominant speech and language disorder (PSP-SL): Characterized by progressive nonfluent aphasia or speech or language disorders

  • PSP with predominant frontal presentation (PSP-F): Characterized by frontotemporal degeneration with cognitive impairment or behavioral change

  • PSP with predominant corticobasal syndrome (PSP-CBS): Characterized by corticobasal syndrome, causing profound asymmetric symptoms

  • PSP with predominant cerebellar ataxia (PSP-C): Characterized by cerebellar signs

  • PSP with predominant postural instability (PSP-PI): Characterized by postural instability

In forms other than Richardson syndrome (nonclassical forms [called atypical parkinsonism]), ophthalmoplegia may be delayed by years.

The different clinical types of progressive supranuclear palsy have different characteristics (eg, parkinsonian features, corticobasal syndrome, speech problems, frontotemporal degenerative signs, akinesia, freezing of gait).

Symptoms and signs references

Diagnosis of Progressive Supranuclear Palsy

  • Clinical evaluation

MRI is usually done to exclude other disorders. In advanced cases, MRI shows a characteristic decrease in midbrain size that is best seen on midsagittal views and that causes the midbrain to be shaped like a hummingbird or emperor penguin. On axial views, the midbrain may resemble a morning glory (2 Diagnosis references Progressive supranuclear palsy is a rare, degenerative central nervous system disorder that progressively impairs voluntary eye movements and causes bradykinesia, muscular rigidity with progressive... read more Diagnosis references ).

Diagnosis references

Treatment of Progressive Supranuclear Palsy

  • Supportive care

Treatment of PSP focuses on relieving symptoms; antiparkinsonian medications may be used, but they relieve symptoms much less effectively than they do in patients with Parkinson disease. Physical and occupational therapy may help improve mobility and function and reduce the risk of falls.

Because patients with PSP have a poor prognosis, they and their families are encouraged to discuss advanced cared directives early. Expected decline in PSP involves early cognitive impairment with dysphagia. Aspiration is a common cause of death.

Key Points

  • The first symptom of classic progressive supranuclear palsy may be difficulty looking up or down without moving the neck or difficulty climbing up and down stairs.

  • The different clinical types of progressive supranuclear palsy have different characteristics (eg, parkinsonian features, corticobasal syndrome, speech problems, frontotemporal degenerative signs, akinesia, freezing of gait).

  • Diagnose based on symptoms, but do MRI to exclude other disorders.

  • Focus on relieving symptoms; consider levodopa and/or amantadine to relieve rigidity, and prescribe physical and occupational therapy.

Drugs Mentioned In This Article

Drug Name Select Trade
INBRIJA, Larodopa
GOCOVRI, Osmolex ER, Symmetrel
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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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