Childhood-onset benign chorea with striatal involvement

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:494541OMIM:616922G25.5
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Childhood-onset benign chorea with striatal involvement is a rare neurological condition that begins in childhood and causes involuntary, irregular movements of the body. The word 'chorea' comes from the Greek word for dance, and it describes the flowing, unpredictable movements that are the main feature of this condition. These movements most often affect the arms, legs, and face, and they happen without the person meaning to move. The brain region called the striatum — a part of the brain that helps control movement — is specifically affected in this condition. Despite the word 'benign' in its name, this condition can still significantly affect a child's daily life, including their ability to write, speak clearly, and participate in school activities. The movements tend to be non-progressive, meaning they do not necessarily get worse over time, which is why the condition is considered relatively benign compared to other movement disorders. Some children may also experience mild difficulties with coordination or balance. Treatment focuses on managing symptoms rather than curing the underlying cause. Medications that calm abnormal movement signals in the brain, such as certain anti-seizure or dopamine-blocking drugs, may be used. Physical and occupational therapy can also help children build skills and adapt to their symptoms. Because this is a rare condition, care is usually provided by a specialist in movement disorders or pediatric neurology.

Key symptoms:

Involuntary, flowing, dance-like movements of the arms, legs, or faceDifficulty controlling hand movements, making writing or drawing hardUnsteady or clumsy walkingFacial grimacing or twitchingSlurred or unclear speechDifficulty with fine motor tasks like buttoning clothesMuscle tone that may feel reduced or floppyRestlessness or fidgetingMild problems with coordination and balance

Clinical phenotype terms (7)— hover any for plain English
Abnormal corpus striatum morphologyHP:0010994Striatal T2 hyperintensityHP:0031206Parkinsonism with favorable response to dopaminergic medicationHP:0002548
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Childhood-onset benign chorea with striatal involvement.

View clinical trials →

No actively recruiting trials found for Childhood-onset benign chorea with striatal involvement at this time.

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

Search ClinicalTrials.gov ↗Join the Childhood-onset benign chorea with striatal involvement community →

No specialists are currently listed for Childhood-onset benign chorea with striatal involvement.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 Childhood-onset benign chorea with striatal involvement.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Childhood-onset benign chorea with striatal involvementForum →

No community posts yet. Be the first to share your experience with Childhood-onset benign chorea with striatal involvement.

Start the conversation →

Latest news about Childhood-onset benign chorea with striatal involvement

No recent news articles for Childhood-onset benign chorea with striatal involvement.

Follow this condition to be notified when news becomes available.

Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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.What is the most likely cause of my child's movements, and should we do genetic testing?,Will the movements get worse over time, or are they likely to stay the same?,What medications are available, and what are the risks and benefits of each?,What therapies would you recommend, and how often should my child attend?,What accommodations should we request from my child's school?,Are there any clinical trials or research studies we could consider joining?,Should other family members be evaluated or tested for a similar condition?

Common questions about Childhood-onset benign chorea with striatal involvement

What is Childhood-onset benign chorea with striatal involvement?

Childhood-onset benign chorea with striatal involvement is a rare neurological condition that begins in childhood and causes involuntary, irregular movements of the body. The word 'chorea' comes from the Greek word for dance, and it describes the flowing, unpredictable movements that are the main feature of this condition. These movements most often affect the arms, legs, and face, and they happen without the person meaning to move. The brain region called the striatum — a part of the brain that helps control movement — is specifically affected in this condition. Despite the word 'benign' in

At what age does Childhood-onset benign chorea with striatal involvement typically begin?

Typical onset of Childhood-onset benign chorea with striatal involvement is childhood. Age of onset can vary across affected individuals.