Progressive supranuclear palsy-corticobasal syndrome

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ORPHA:240103OMIM:260540G23.1
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39Specialists8Treatment centers

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Overview

Progressive supranuclear palsy-corticobasal syndrome (PSP-CBS) is a rare brain disorder that belongs to a group of conditions called tauopathies, where an abnormal protein called tau builds up in brain cells and causes them to die. This condition combines features of two related diseases: progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). It is sometimes referred to as PSP with corticobasal features or an overlap syndrome between PSP and CBS. People with PSP-CBS typically develop problems with movement, balance, and thinking in their 60s or 70s. Common symptoms include stiffness and clumsiness that is worse on one side of the body (asymmetric), difficulty controlling eye movements (especially looking downward), problems with balance leading to falls, slowed movements, speech difficulties, and progressive cognitive decline. The affected person may also experience a phenomenon called "alien limb," where one hand seems to move on its own. There is currently no cure for PSP-CBS, and treatment focuses on managing symptoms and maintaining quality of life. Physical therapy, occupational therapy, and speech therapy play important roles. Some medications used for Parkinson's disease, such as levodopa, may be tried but usually provide limited benefit. The disease is progressive, meaning symptoms worsen over time. Research into tau-targeting therapies and other disease-modifying treatments is ongoing, offering hope for future breakthroughs.

Also known as:

Key symptoms:

Stiffness and rigidity worse on one side of the bodyDifficulty looking up or down with the eyesFrequent falls, especially backwardSlowed movementsClumsiness or loss of coordination in one handAlien limb phenomenon (a hand that seems to move on its own)Difficulty speaking or slurred speechTrouble swallowingProblems with thinking, planning, and memoryPersonality or behavior changesDifficulty writingMuscle jerking or tremor in one limbPoor balanceDepression or apathy

Clinical phenotype terms (30)— hover any for plain English
ApraxiaHP:0002186Progressive extrapyramidal muscular rigidityHP:0007158Slow saccadic eye movementsHP:0000514Diminished movementHP:0002374Vertical supranuclear gaze palsyHP:0000511Focal dystoniaHP:0004373Frontal release signsHP:0000743
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-corticobasal syndrome.

View clinical trials →

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

New trials open frequently. Follow this disease to get notified.

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

Specialists

Showing 25 of 39View all specialists →
BM
benzi Kluger, MD
AURORA, CO
Specialist
PI on 1 active trial
NP
Nupur Ghoshal, MD, PhD
SAINT LOUIS, MO
Specialist
PI on 1 active trial
HP
Hector Rieiro, PhD
Specialist
PI on 1 active trial
BM
Benzi M Kluger, MD, MS
AURORA, CO
Specialist
PI on 1 active trial
BM
Bradley Boeve, MD
ROCHESTER, MN
Specialist
PI on 3 active trials
BM
Bruno Dubois, MD
Specialist
PI on 1 active trial
AP
Adam Boxer, MD, PhD
SAN FRANCISCO, CA
Specialist
PI on 12 active trials
DM
David Irwin, MD
Specialist
PI on 1 active trial
FM
Florence Pasquier, MD
Specialist
PI on 1 active trial
AM
Audrey Gabelle, MD
Specialist
PI on 1 active trial1 Progressive supranuclear palsy-corticobasal syndrome publication
EM
Eloi Magnin, MD
Specialist
PI on 1 active trial
FM
François Sellal, MD
Specialist
PI on 1 active trial
TP
Tyler Roncero, Ph.D
Specialist
PI on 1 active trial
RP
Renè Gonin, PhD
Specialist
PI on 1 active trial
HM
Howard Chertkow, MD
Specialist
PI on 1 active trial
HM
Hakan Gurvit, MD
Specialist
PI on 1 active trial
AP
Adam L. Boxer, M.D., Ph.D.
SAN FRANCISCO, CA
Specialist
PI on 2 active trials
LP
Laura Andres-Martin, PhD
Specialist
PI on 1 active trial

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-corticobasal syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Progressive supranuclear palsy-corticobasal syndrome

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Caregiver Resources

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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.What subtype of PSP do I have, and how does PSP-CBS differ from other forms?,What is the expected rate of progression in my case?,Are there any clinical trials or experimental treatments I might be eligible for?,What therapies (physical, occupational, speech) should I start now to maintain function?,How should we plan for future care needs, including swallowing difficulties and mobility loss?,Should my family members consider genetic testing?,What resources or support groups are available for patients and caregivers?

Common questions about Progressive supranuclear palsy-corticobasal syndrome

What is Progressive supranuclear palsy-corticobasal syndrome?

Progressive supranuclear palsy-corticobasal syndrome (PSP-CBS) is a rare brain disorder that belongs to a group of conditions called tauopathies, where an abnormal protein called tau builds up in brain cells and causes them to die. This condition combines features of two related diseases: progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). It is sometimes referred to as PSP with corticobasal features or an overlap syndrome between PSP and CBS. People with PSP-CBS typically develop problems with movement, balance, and thinking in their 60s or 70s. Common symptoms include stif

How is Progressive supranuclear palsy-corticobasal syndrome inherited?

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

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

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

Which specialists treat Progressive supranuclear palsy-corticobasal syndrome?

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