Progressive supranuclear palsy-predominant parkinsonism syndrome

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ORPHA:240085OMIM:260540G23.1
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1Specialists8Treatment centers

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Overview

Progressive supranuclear palsy-predominant parkinsonism syndrome (PSP-P) is a rare brain disorder that belongs to the family of progressive supranuclear palsy conditions. It is sometimes called PSP-parkinsonism because its early symptoms closely resemble Parkinson's disease, which can make it harder to diagnose at first. In PSP-P, certain brain cells gradually break down due to an abnormal buildup of a protein called tau. This leads to problems with movement, balance, and other body functions. People with PSP-P typically experience slowness of movement, stiffness, and tremor — symptoms that look very much like typical Parkinson's disease. They may initially respond to levodopa, a common Parkinson's medication, but this response usually fades over time. As the disease progresses, patients may develop difficulty with eye movements (especially looking up and down), problems with balance and frequent falls, speech and swallowing difficulties, and changes in thinking and behavior. Compared to the classic form of PSP (called Richardson syndrome), PSP-P tends to progress more slowly and patients may live longer. There is currently no cure for PSP-P. Treatment focuses on managing symptoms and maintaining quality of life. Medications used for Parkinson's disease may provide some temporary relief. Physical therapy, occupational therapy, and speech therapy are important parts of care. Research into tau-targeting therapies and other disease-modifying treatments is ongoing, offering hope for future breakthroughs.

Also known as:

Key symptoms:

Slowness of movement (bradykinesia)Muscle stiffness and rigidityTremor, often resembling Parkinson's diseaseDifficulty with balance and frequent fallsTrouble moving the eyes, especially looking up or downSlurred or slow speechDifficulty swallowingChanges in personality or behaviorProblems with thinking and memoryTrouble with handwritingFacial expression becoming less animated (masked face)Sleep disturbancesDepression or apathyNeck stiffness or abnormal posture

Clinical phenotype terms (20)— hover any for plain English
Axial muscle stiffnessHP:0006921Neuromuscular dysphagiaHP:0002068Parkinsonism with favorable response to dopaminergic medicationHP:0002548Impaired visuospatial constructive cognitionHP:0010794Vertical supranuclear gaze palsyHP:0000511Abnormal saccadic eye movementsHP:0000570
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Late onset

Begins later in life, typically after age 50

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Progressive supranuclear palsy-predominant parkinsonism syndrome.

View clinical trials →

No actively recruiting trials found for Progressive supranuclear palsy-predominant parkinsonism syndrome at this time.

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Search ClinicalTrials.gov ↗Join the Progressive supranuclear palsy-predominant parkinsonism syndrome community →

Specialists

1 foundView all specialists →
FP
Fabrizio Stocchi, MD, PHD
Specialist
PI on 2 active trials

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Progressive supranuclear palsy-predominant parkinsonism syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.How can you be sure this is PSP-P and not Parkinson's disease?,Should I try levodopa, and how will we know if it is helping?,What physical therapy and exercise programs do you recommend to reduce falls?,Are there any clinical trials for PSP that I might be eligible for?,When should I see a speech therapist about swallowing or speech problems?,What should I plan for as the disease progresses, and when should we discuss advance care planning?,Is genetic testing recommended for me or my family members?

Common questions about Progressive supranuclear palsy-predominant parkinsonism syndrome

What is Progressive supranuclear palsy-predominant parkinsonism syndrome?

Progressive supranuclear palsy-predominant parkinsonism syndrome (PSP-P) is a rare brain disorder that belongs to the family of progressive supranuclear palsy conditions. It is sometimes called PSP-parkinsonism because its early symptoms closely resemble Parkinson's disease, which can make it harder to diagnose at first. In PSP-P, certain brain cells gradually break down due to an abnormal buildup of a protein called tau. This leads to problems with movement, balance, and other body functions. People with PSP-P typically experience slowness of movement, stiffness, and tremor — symptoms that l

How is Progressive supranuclear palsy-predominant parkinsonism syndrome inherited?

Progressive supranuclear palsy-predominant parkinsonism syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Progressive supranuclear palsy-predominant parkinsonism syndrome typically begin?

Typical onset of Progressive supranuclear palsy-predominant parkinsonism syndrome is late onset. Age of onset can vary across affected individuals.

Which specialists treat Progressive supranuclear palsy-predominant parkinsonism syndrome?

1 specialists and care centers treating Progressive supranuclear palsy-predominant parkinsonism syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.