Progressive Supranuclear Palsy
A rare brain disorder affecting balance, eye movement, and thinking
Quick Facts
- Type: Progressive brain (neurodegenerative) disorder
- Hallmark: Falls and trouble moving the eyes
- Often mistaken for: Parkinson disease
- Typical onset: Usually after age 60
Overview
Progressive supranuclear palsy (PSP) is a rare brain disorder that worsens over time and affects movement, balance, eye control, speech, swallowing, and thinking. It belongs to a group of conditions called atypical parkinsonian disorders because, like Parkinson disease, it causes stiffness and slowness, but it follows a different course and does not respond well to the usual Parkinson medications.
The word supranuclear refers to the part of the brain, above the nerve centers that move the eyes, that becomes damaged, which is why difficulty moving the eyes, especially looking up and down, is a characteristic feature. PSP is caused by the buildup of an abnormal form of a protein called tau in brain cells. It usually begins after age 60 and gradually reduces mobility and independence, though supportive care can meaningfully improve safety and quality of life.
Symptoms
Symptoms develop gradually and worsen over time. Common features include:
- Loss of balance and frequent falls, often backward, sometimes early in the disease
- Difficulty moving the eyes, especially looking down, leading to problems with stairs and reading
- Stiffness and slowness of movement
- Changes in speech, such as slurred or quiet speech
- Difficulty swallowing
- Changes in mood, thinking, and behavior, including apathy or trouble with planning
- A fixed, staring facial expression
Unexplained falls and a tendency to fall backward are often among the earliest clues.
Causes
Progressive supranuclear palsy is caused by the gradual loss of nerve cells in specific areas of the brain that control movement, balance, and eye movements. This cell damage is associated with the abnormal buildup of a protein called tau, which clumps inside brain cells and interferes with their function.
Why this happens is not well understood. The vast majority of cases occur sporadically, without a clear family history, and PSP is not considered an inherited disease in most people. It is not caused by lifestyle, infection, or anything a person did, and it cannot be passed to others.
Risk Factors
- Older age, with most cases beginning after 60
- Slightly more common in men
Unlike many conditions, PSP has few identified risk factors, and there is no proven way to prevent it. A family history is uncommon.
Diagnosis
There is no single test for PSP, so diagnosis is based on the pattern of symptoms and the exclusion of other conditions:
- Neurological examination: Assessing balance, eye movements, stiffness, and thinking, with the eye-movement problems being especially telling.
- MRI of the brain: May show shrinkage in specific regions and helps rule out other causes such as stroke.
- Response to Parkinson medication: A limited or absent response can help distinguish PSP from Parkinson disease.
- Ongoing follow-up: Because early PSP can resemble Parkinson disease, the diagnosis often becomes clearer over time.
Treatment
There is no cure or treatment that stops PSP from progressing, so care focuses on managing symptoms and maintaining safety and independence:
- Physical therapy: Balance and gait training, and strategies to reduce falls.
- Occupational therapy: Home safety adjustments, walking aids such as weighted walkers, and tools to support daily tasks.
- Speech and swallowing therapy: Techniques to keep communication and safe eating for as long as possible.
- Medications: Some people get partial, temporary benefit from Parkinson medications, and other drugs may help specific symptoms.
- Supportive measures: Special glasses for downward gaze, and planning for future care needs.
A coordinated team approach offers the most benefit.
Prevention
There is no known way to prevent progressive supranuclear palsy, since its cause is not well understood and it is not driven by lifestyle. Care instead focuses on reducing the impact of symptoms:
- Take steps to prevent falls, such as removing trip hazards and using recommended walking aids
- Maintain regular therapy to preserve mobility and communication
- Plan ahead for swallowing and care needs
When to See a Doctor
See a doctor if you or a loved one has unexplained falls, increasing stiffness, trouble moving the eyes, or changes in speech, swallowing, or thinking. Early evaluation helps clarify the diagnosis and connect you with supportive care. Seek prompt care for:
- A fall causing a head injury or loss of consciousness
- Choking or repeated difficulty swallowing
- Sudden, severe changes in movement or awareness
Frequently Asked Questions
How is PSP different from Parkinson disease?
Both cause stiffness and slowness, but PSP typically causes early falls (often backward), trouble moving the eyes (especially looking down), and a poor or short-lived response to Parkinson medications. The diagnosis often becomes clearer as symptoms progress.
Is progressive supranuclear palsy inherited?
In most people it occurs sporadically, without a clear family history, and is not considered an inherited disease. Rare familial cases exist, but the great majority arise on their own.
Is there a cure for PSP?
There is currently no cure and no treatment that stops it from progressing. Care focuses on managing symptoms and maintaining safety with physical, occupational, and speech therapy, plus measures to prevent falls.
Why do people with PSP fall so often?
PSP damages the brain regions controlling balance and eye movement, which makes it hard to stay steady and to see hazards, especially when looking down. Falls, often backward, are frequently one of the earliest signs.
What causes progressive supranuclear palsy?
It is associated with the abnormal buildup of a protein called tau, which damages nerve cells in areas controlling movement, balance, and eye control. Why this buildup occurs is not well understood.
References
- National Institute of Neurological Disorders and Stroke (NINDS). Progressive Supranuclear Palsy.
- Mayo Clinic. Progressive supranuclear palsy.
- National Organization for Rare Disorders (NORD). Progressive Supranuclear Palsy.
- MedlinePlus, U.S. National Library of Medicine. Progressive supranuclear palsy.