ConditionBrain & nerves
Progressive Supranuclear Palsy (PSP)
Progressive supranuclear palsy (PSP) is a rare, progressive brain disease that affects movement, balance, eye movements, speech, swallowing and thinking. It is caused by a buildup of the protein tau in parts of the brainstem and basal ganglia. The hallmark signs are unexplained falls, often backward, in the first year, and difficulty moving the eyes up and down. It usually begins in the 60s and is often first mistaken for Parkinson's disease. There is no cure yet, but therapy and support help maintain safety and quality of life.
Also called: PSP, Steele-Richardson-Olszewski syndrome, Richardson syndrome
At a glance
- What it is
- A rare neurodegenerative disease caused by abnormal tau protein in the brain.
- How common
- About 5 to 7 people per 100,000; usually starts after age 60.
- Key signs
- Early falls, stiff upright posture, slowed or limited up-and-down eye movements, slurred speech, swallowing difficulty.
- Often mistaken for
- Parkinson's disease, which responds better to medicines.
- Diagnosis
- Clinical examination by a neurologist; MRI can support it.
- Care
- Physical, speech and occupational therapy, fall prevention, swallowing support, palliative care.
When to get help
Call 911 or go to the ER
- Choking episodes with difficulty breathing
- Fever, cough and breathlessness after coughing during meals (possible aspiration pneumonia)
- A fall with a head injury, especially in someone taking blood thinners, or followed by drowsiness or confusion: call 911 or your local emergency number
See a doctor within a day or two
- Swallowing becoming much harder, with weight loss or frequent coughing at meals
- Sudden worsening of confusion, walking or speech
Book a routine appointment
- Unexplained falls, especially backward, in someone over 50
- Difficulty looking down, for example when walking downstairs or eating
- Slurred speech, a staring expression, reduced blinking and personality changes such as apathy
- A diagnosis of Parkinson's disease that is not responding well to levodopa, with early falls
What happens in PSP
In PSP, the protein tau forms clumps inside nerve cells and support cells in the brainstem, basal ganglia and frontal lobes. These areas control balance, posture, eye movements, swallowing, speech and behavior. The term "supranuclear" refers to damage above the nerve centers that move the eyes, so the eye muscles themselves are healthy but the brain cannot command them properly.
The cause is not known. PSP is usually not inherited, although some genetic variations increase susceptibility.
Symptoms
Balance and movement
- unexplained falls, often backward, typically in the first year
- stiffness of the neck and trunk, with an upright or backward-leaning posture
- slowness of movement
- a broad-based, unsteady walk; sudden "rocket sign" of standing up quickly and impulsively
Eyes
- difficulty moving the eyes up and down, especially down (vertical gaze palsy), making stairs and eating difficult
- blurred or double vision
- reduced blinking, a staring or surprised expression
- light sensitivity and difficulty opening the eyelids
Speech and swallowing
- slow, slurred, low-pitched speech
- difficulty swallowing (dysphagia), coughing and choking at meals
Thinking and behavior
- slowed thinking and difficulty planning and problem-solving
- apathy and loss of interest
- impulsivity, irritability and emotional outbursts (laughing or crying inappropriately)
- depression
Variants
Not everyone has the classic picture (Richardson syndrome). PSP variants can look like Parkinson's disease (PSP-parkinsonism), cause mainly freezing of gait, speech and language problems, or behavior changes like frontotemporal dementia.
PSP vs Parkinson's disease
| Feature | PSP | Parkinson's disease |
|---|---|---|
| Early falls | Common in the first year | Usually later |
| Posture | Upright or leaning back | Stooped forward |
| Tremor | Uncommon | Common, at rest |
| Eye movements | Up and down gaze limited | Usually normal |
| Response to levodopa | Little or brief | Good |
| Symmetry | Usually symmetrical | Usually starts on one side |
| Progression | Faster | Slower |
PSP can also be confused with multiple system atrophy, corticobasal degeneration and Lewy body dementia.
Diagnosis
There is no single test. A neurologist, ideally a movement disorder specialist, diagnoses PSP from the pattern of symptoms and examination, especially eye movements and balance. Supporting tests:
- MRI: may show shrinkage of the midbrain (the "hummingbird" or "penguin" sign) and other patterns.
- DaTscan shows reduced dopamine activity but does not distinguish PSP from Parkinson's disease.
- Trial of levodopa: a poor response supports PSP.
- Swallowing and speech assessments.
Diagnosis is often made a few years after symptoms start, because early signs are subtle.
Treatment and care
No treatment currently slows or stops PSP; clinical trials are testing therapies aimed at tau. Care focuses on symptoms, safety and quality of life:
- Medicines: levodopa may help stiffness and slowness a little in some people; antidepressants for mood; botulinum toxin for eyelid spasms or neck stiffness.
- Physical therapy: balance training, walking aids (weighted walkers), and fall prevention.
- Occupational therapy: home modifications, grab rails, bathroom safety, adaptive tools.
- Speech and language therapy: communication strategies and swallowing techniques; diet texture changes.
- Eye care: lubricating drops for dry eyes; prism glasses for some.
- Nutrition: dietitian support; a feeding tube may be discussed in advanced swallowing problems.
- Palliative care and advance care planning early in the course.
- Caregiver support and respite.
Outlook
PSP progresses steadily. Most people need a wheelchair within several years. Average survival is about 6 to 8 years from symptom onset, with wide variation. The most common causes of death are pneumonia from aspiration and complications of falls. Good multidisciplinary care can improve comfort and safety.
Frequently asked questions
What are the first signs of progressive supranuclear palsy?
Early signs often include unexplained falls, especially backward, unsteadiness, stiffness, slowed movements, blurred vision or difficulty looking down, slurred speech, and personality changes such as apathy or irritability. Symptoms usually start after age 60.
How is PSP different from Parkinson's disease?
PSP causes early falls, an upright stiff posture, difficulty moving the eyes up and down, and usually little or no tremor. It responds poorly to Parkinson's medicines and progresses faster. Parkinson's disease usually starts on one side with tremor and responds well to levodopa.
What is the life expectancy with PSP?
On average, people live about 6 to 8 years after symptoms start, though this varies. Complications such as aspiration pneumonia from swallowing problems and injuries from falls are the main causes of death. Good care can reduce these risks.
Is PSP hereditary?
PSP is usually not inherited. Most cases occur in people with no family history. Certain common variations in the tau gene slightly increase the risk, and very rare families have inherited forms, but children of a person with PSP are generally not at significantly increased risk.
Sources
This guide was written from current patient and clinical guidance, including:
- National Institute of Neurological Disorders and Stroke: Progressive supranuclear palsy
- CurePSP: About PSP
- International Parkinson and Movement Disorder Society: MDS clinical diagnostic criteria for PSP
- MedlinePlus: Progressive supranuclear palsy
- National Organization for Rare Disorders (NORD): Progressive supranuclear palsy