Progressive Supranuclear Palsy
What is Progressive Supranuclear Palsy?
Overview
Progressive Supranuclear Palsy (PSP) is a rare and progressive neurodegenerative disorder that primarily affects movement and balance control. PSP is considered an atypical parkinsonism syndrome.
The condition is characterized by a variety of symptoms, including:
- Impaired balance and frequent falls: Individuals with PSP often experience difficulty maintaining their balance and are prone to unexplained falls.
- Abnormal eye movements: PSP is known for impairing voluntary eye movements, particularly difficulty looking up and down. This can lead to a distinctive gaze impairment, often described as “supranuclear vertical gaze palsy.”
- Parkinsonian symptoms: PSP shares some motor symptoms with Parkinson’s disease, including bradykinesia (slowness of movement), muscle rigidity, and tremors.
- Cognitive changes: Some people with PSP may develop cognitive impairment and behavioral changes, such as apathy and emotional lability.
This is distinguished from Parkinson’s disease (PD), which is caused by a build-up of the toxic form of alpha-synuclein. Other diseases that are also tauopathies include corticobasal degeneration (CBD) and frontotemporal dementia (FTD).
Progressive supranuclear palsy should be considered when a patient with parkinsonism has frequent, early falls; dementia; difficulty moving the eyes (especially in the vertical direction); eyelid opening apraxia (trouble coordinating the eyelids) and emotional lability and freezing of gait.
Freezing refers to patients having difficulty initiating gait, the feet appearing to be stuck to the floor, then improving after a few steps. Freezing is often brought on by turning, walking in crowded areas, or walking through doorways. In some patients the primary symptom is gait freezing without associated symptoms, called primary freezing of gait (FOG). FOG is felt to be caused by various conditions and not believed to represent a discrete disease. FOG can be caused by PSP, but also vascular parkinsonism, normal pressure hydrocephalus, and Parkinson’s disease.
Who is Affected?
Progressive supranuclear palsy typically affects people over the age of 60, though symptoms can begin earlier. It affects men and women equally and is not inherited in most cases. While rare, PSP impacts thousands of people in the U.S. each year, often leading to significant physical and emotional challenges.
Progressive Supranuclear Palsy Symptoms & Causes
Symptoms
Progressive supranuclear palsy symptoms usually develop gradually and worsen over time. Common signs include:
- Frequent, unexplained falls, often backward.
- Vertical gaze palsy (difficulty moving the eyes up or down).
- Freezing of gait and difficulty initiating walking.
- Stiffness and slowness of movement (similar to Parkinson’s disease).
- Changes in speech and swallowing (slurred speech, difficulty swallowing).
- Cognitive decline (slowed thinking, difficulty planning, and decision-making).
- Behavioral changes, including apathy and emotional outbursts.
- Eyelid apraxia (difficulty opening or closing the eyes voluntarily).
Causes
Progressive supranuclear palsy is caused by the abnormal accumulation of tau protein in brain regions responsible for movement, balance, and thinking. This accumulation damages and kills nerve cells, leading to the symptoms of PSP.
Unlike some neurological conditions, PSP is not usually inherited. The exact reason why tau builds up in PSP is still under investigation, though genetics and environmental factors may play a role.
When to See a Doctor
Early diagnosis can help ensure proper management and support. Seek medical evaluation if you or a loved one experiences:
- Sudden changes in balance, walking, or speech
- Frequent, unexplained falls
- Difficulty looking up or down
- Unexplained emotional changes or cognitive decline
Progressive Supranuclear Palsy Diagnosis
There are various findings on brain MRI that can help establish the diagnosis of progressive supranuclear palsy in combination with the clinical findings. This includes midbrain atrophy (shrinking) and bright signals (hyperintensities) in the pontine tegmentum (a part of the brainstem). Because these findings can be subtle, especially early in the course of the disease, our movement disorders specialists will personally review all imaging in patients suspected of having PSP, freezing of gait, or atypical parkinsonism. DaTscan cannot distinguish between PSP and PD but can help distinguish between Alzheimer’s disease (AD) and parkinsonian conditions.
Progressive Supranuclear Palsy Treatment & Outcomes
Treatment Options
Unfortunately there is no cure for progressive supranuclear palsy, however, symptoms may improve marginally and temporarily with:
Parkinson’s Medications (Levodopa)
May help some motor symptoms, though the response is often limited.
Physical Therapy
To improve balance, gait, and prevent falls.
Speech Therapy
To address swallowing and communication difficulties.
Occupational Therapy
To maximize independence in daily activities.
There are small studies that are enrolling to modify the tau protein but are in very early stages. Research trials are ongoing to target tau protein and explore disease-modifying therapies.
Patient Outcomes
Progressive supranuclear palsy is progressive, meaning symptoms worsen over time. While the average life expectancy after diagnosis is 6–10 years, supportive therapies can significantly improve quality of life, mobility, and independence for as long as possible.
Managing Progressive Supranuclear Palsy
Living with progressive supranuclear palsy requires a multidisciplinary approach:
- Physical and speech therapy to maintain function
- Fall prevention strategies at home
- Nutritional support to address swallowing issues
- Assistive devices (walkers, wheelchairs) for mobility
- Support groups and counseling for patients and caregivers
Meet our Expert Specialists & Surgeons
Experience Compassionate, Expert Care
At Pacific Neuroscience Institute, our movement disorder specialists have expertise in diagnosing and managing progressive supranuclear palsy and other atypical parkinsonism disorders. We understand the challenges of living with PSP. Our team provides personalized care, combining medical expertise, therapy, and support services to enhance quality of life for patients and their families.
Learn More About Progressive Supranuclear Palsy
On average, individuals live 6–10 years after diagnosis, though this varies based on overall health and symptom severity.
In advanced stages, patients often experience severe swallowing difficulties, profound mobility limitations, and increased risk of infections.
PSP is caused by tau protein buildup in the brain. The exact trigger is unknown but may involve genetic and environmental factors.
PSP typically progresses through stages: early balance problems, worsening eye movement and gait issues, increasing difficulty with speech and swallowing, and advanced immobility and cognitive decline.
Yes. While treatments can manage symptoms, PSP is progressive and ultimately life-limiting.
Resources
Get Expert Care from Leading Specialists
If you or a loved one is experiencing symptoms of PSP, early evaluation by a movement disorder specialist is essential. Our team provides comprehensive care and support to help patients navigate this complex condition.
Written and reviewed by:
The Pacific Neuroscience medical and editorial team
We are a highly specialized team of medical professionals with extensive neurological and cranial disorder knowledge, expertise and writing experience.
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