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
Variable
Can be inherited in different ways depending on the underlying gene
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Childhood-onset benign chorea with striatal involvement.
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Specialists
View all specialists →No specialists are currently listed for Childhood-onset benign chorea with striatal involvement.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA 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.
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Caregiver Resources
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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.