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Progressive Supranuclear Palsy

Practice Essentials

Progressive supranuclear palsy (PSP) is a neurodegenerative disease (see the image below) whose characteristics include supranuclear, initially vertical, gaze dysfunction accompanied by extrapyramidal symptoms and cognitive dysfunction. The disease usually develops after the sixth decade of life, and the diagnosis is purely clinical.

Sagittal T1-weighted image shows atrophy of midbra

Sagittal T1-weighted image shows atrophy of midbrain, preservation of pontine volume, and atrophy of the tectum, suggestive of progressive supranuclear palsy (Steele-Olszewski-Richardson disease).

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Signs and symptoms

The onset of PSP is insidious and usually includes a prolonged phase marked by the following symptoms:






Patients also experience subtle personality changes, memory problems, and pseudobulbar symptoms, which are often more evident to the family than to the patient. The initial symptoms can often involve unexplained imbalance or falls.

The cardinal manifestations of PSP are as follows:

Supranuclear ophthalmoplegia

Pseudobulbar palsy

Prominent neck dystonia


Behavioral, cognitive, and gait disturbances that cause imbalance

Frequent falls/impaired postural reflexes

Findings on physical examination can include the following:

Poor postural reflexes, axial rigidity greater than appendicular rigidity, and dysarthria (monotone with slight hypophonic quality)

Absence of cogwheeling or tremor

Widely based and unstable gait

Bradykinesia with masked facies and a startled expression

Retrocollis may be present

Visual signs and symptoms

Slow vertical saccades and square wave jerks on ocular examination (early signs)

Supranuclear ophthalmoplegia (classic gaze palsy in PSP)

Downgaze typically involved before upgaze

Improvement in supranuclear vertical gaze limitation after extravolitional pathway activation with the vestibular ocular reflex (VOR) or the Bell phenomenon

Nearly continuous square wave jerks commonly observed with fixation

Impairment of convergence eye movements (may cause diplopia)

Eyelid retraction, eyelid opening or closing apraxia, blepharospasm, or lid lag

Complete ophthalmoparesis in advanced PSP

Cognitive symptoms

Slowed cognitive processing, sequencing and planning difficulties, mild memory difficulty, and apathy (generally more prominent in late disease)

High apathy scores coupled with low agitation and anxiety scale scores on Neuropsychiatric Inventory testing

See Clinical Presentation for more detail.


The diagnosis of PSP is clinical. Key features typically develop over time. Participants in a National Institute of Neurological Disorders and Stroke (NINDS)/Society for PSP conference have formulated and validated clinical research criteria for the diagnosis of PSP.
In this system, criteria for possible PSP are as follows:

Gradually progressive disorder with onset at age 40 years or older

Either vertical supranuclear palsy or both slowing of vertical saccades and prominent postural instability with falls in the first year of onset

No evidence of other diseases that can explain the clinical features

Criteria for probable PSP are vertical supranuclear palsy with prominent postural instability, falls in the first year of onset, and other features of possible PSP, as follows:

Abnormal neck posture, especially retrocollis

Poor or absent response of parkinsonism to levodopa therapy

Early dysphagia and dysarthria

Early cognitive impairment with at least 2 of the following: apathy, abstract thought impairment, decreased verbal fluency, imitation behavior, or frontal release signs

Criteria for definite PSP are as follows:

History of probable or possible PSP

Histopathologic evidence that is typical of the disease

The workup in patients with suspected PSP is directed principally at eliminating other diagnoses (eg, Whipple PCR to eliminate possible Whipple disease). MRI offers little help in the early stages of PSP, but may reveal the following abnormalities in some advanced cases

Atrophy of the midbrain (see the image below) with cisternal and ventricular dilatation

Thinning of the quadrigeminal plate

Dilation of the third ventricle

A nonspecific finding of increase in proton density images in the periaqueductal gray matter, compatible with gliotic changes

Sleep studies in patients with PSP show the following abnormalities, although these are not specific for PSP:

Diminished total sleep time

Increased awakenings

Progressive loss of rapid-eye-movement (REM) sleep

Decreased REM–to–non-REM (NREM) quotient

See Workup for more detail.


No medication is effective in halting the progression of PSP; however, medications that may provide modest symptomatic improvement include the following:

Dopamine agonists

Tricyclic antidepressants


OnabotulinumtoxinA: May be useful for rigidity (nuchal rigidity in particular) and dystonia (eg, blepharospasm, bruxism, and focal limb dystonia)

Methylcellulose or methyl alcohol eyedrops: For relief of chronic conjunctivitis from reduced blink rate

Physical therapy and rehabilitation medicine involvement may help maximize ambulation safety and facilitate instruction in the use of a walker, wheelchair, or other aids.

See Treatment and Medication for more detail.

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