What's progressive supranuclear palsy?
Progressive supranuclear palsy (PSP) is a chronic neurodegenerative disease, a form of atypical parkinsonism in which an abnormal form of the protein tau (4-repeat tau) builds up in the brain. The classic form, Richardson syndrome, is defined by a downward-predominant inability to move the eyes voluntarily (vertical supranuclear gaze palsy), early loss of balance with backward falls, stiffness of the trunk, slowed movement that responds poorly to Parkinson medication, and a frontal pattern of thinking and mood change. PSP is sporadic; no causal gene is confirmed here (MAPT-linked familial tauopathy is a separate, rare entity). There is no treatment that changes the underlying disease; care is supportive.
| Also indexed as | ORPHA:683, MONDO:0019037 |
|---|---|
| Features mapped | 16 |
| Treatments mapped | 4 |
| Published sources | 11 |
| Last reviewed | 2026-08-04 |
Signs and symptoms
Supranuclear gaze palsy
a loss of voluntary control of eye movements, most prominently affecting downward gaze, is a defining feature of progressive supranuclear palsy.
Supranuclear ophthalmoplegia
difficulty moving the eyes, particularly up and down, is one of the features that typically characterizes progressive supranuclear palsy.
Vertical supranuclear gaze palsy
The hallmark sign is a vertical supranuclear gaze palsy: the brain can no longer direct voluntary eye movements, most noticeably looking downward, even though the eyes themselves are intact. This makes stairs, eating, and reading difficult.
Falls
Frequent unexplained falls, often backward, are characteristic and an early source of injury.
Dysarthria
slurred or unclear speech (dysarthria) is associated with progressive supranuclear palsy.
Dementia
a subcortical-frontal pattern of cognitive impairment, affecting thinking and behavior, is a defining feature of progressive supranuclear palsy.
Cognitive impairment
Thinking changes follow a frontal pattern: slowed processing, reduced planning and initiative, impulsivity, and apathy, while memory is relatively less affected early on.
Postural instability
Loss of balance (postural instability) appears early and is often the first problem, with a tendency to topple backwards.
Neuronal loss in central nervous system
progressive supranuclear palsy is a neurodegenerative disorder marked by the buildup of a protein called tau, along with glial dysfunction and inflammation in the brain.
Depression
the symptoms of progressive supranuclear palsy span affective domains such as mood, alongside motor, sensory, cognitive, autonomic, and behavioral changes.
Apathy
Apathy, a loss of motivation and interest, is one of the most common behavioral changes in progressive supranuclear palsy. It comes from the disease's effect on the frontal lobes and is part of the illness rather than depression or laziness, though it can look similar.
Unsteady gait
unsteady balance and walking, with falls occurring early, is characteristic of progressive supranuclear palsy.
Tremor
tremor can occur as part of the parkinsonism seen in progressive supranuclear palsy, alongside rigidity, slowed movement, and gait difficulty.
Bradykinesia
Movements slow down (bradykinesia), but unlike Parkinson disease this slowness responds poorly to levodopa.
Rigidity
Stiffness concentrated in the neck and trunk (axial rigidity) is typical, in contrast to the limb-predominant stiffness of Parkinson disease.
Dysphagia
Difficulty swallowing (dysphagia) appears early in progressive supranuclear palsy and tends to worsen over time. It matters because food or liquid going down the wrong way can cause aspiration pneumonia, which is a leading cause of serious illness in PSP, so swallowing is assessed regularly.
How it is diagnosed
Progressive supranuclear palsy
Diagnosed using: clinical diagnosis (autopsy-confirmed).
“The diagnosis of PSP is mainly based on clinical data and is only definitively confirmed at autopsy.”
Progressive supranuclear palsy
Diagnosed using: brain MRI (midbrain atrophy).
“Axial T2-weighted magnetic resonance imaging (MRI) showed midbrain atrophy, as well as the hummingbird sign and morning glory sign, characteristic of PSP.”
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
multidisciplinary care
multidisciplinary care is increasingly recognized as needed for the best outcomes in progressive supranuclear palsy.
Used to help with: Progressive supranuclear palsy.
“It is increasingly recognized that patients with PSP, CBS, and other parkinsonian disorders require multidisciplinary care for optimal outcomes.”
symptomatic therapy
there are no disease-modifying therapies for progressive supranuclear palsy, but targeted treatment of symptoms can improve quality of life.
Used to help with: Progressive supranuclear palsy.
“Despite the lack of effective disease-modifying therapies, targeted treatment of symptoms can improve quality of life for patients with 4-repeat tauopathies.”
support groups and symptomatic therapies
progressive supranuclear palsy currently lacks disease-modifying therapies, but early diagnosis lets a person benefit from the many available symptomatic therapies, support groups, and a growing number of clinical trials.
Used to help with: Progressive supranuclear palsy.
“Although PSP and CBS currently lack disease-modifying therapies, it is important to diagnose them as early as possible so that the patient can benefit from the many available symptomatic therapies, support groups, and a growing number of clinical trials.”
pharmacologic and nonpharmacologic symptom management
symptomatic management of progressive supranuclear palsy, one of the 4-repeat tauopathies, includes both pharmacologic and nonpharmacologic strategies.
Used to help with: Progressive supranuclear palsy.
“This review describes current approaches to symptomatic management of common clinical symptoms in 4-repeat tauopathies with a focus on practical patient management, including pharmacologic and nonpharmacologic…”
How to read the evidence labels
Where this comes from
This guide is built from 11 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
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