Sydenham chorea

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ORPHA:306731I02.0I02.9
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19Specialists8Treatment centers

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Overview

Sydenham chorea, also known as St. Vitus' dance or rheumatic chorea, is a neurological disorder that occurs as a complication of rheumatic fever. Rheumatic fever itself is triggered by a throat infection caused by group A streptococcus bacteria (strep throat). After the infection, the body's immune system mistakenly attacks parts of the brain, particularly a region called the basal ganglia, which helps control movement. This leads to the hallmark symptom of chorea — involuntary, jerky, irregular movements that can affect the face, arms, legs, and trunk. Symptoms typically appear weeks to months after a strep throat infection and can range from mild clumsiness to severe uncontrollable movements. Children may also experience emotional changes such as mood swings, crying spells, anxiety, and difficulty concentrating. Muscle weakness, trouble with handwriting, slurred speech, and difficulty walking are also common. In some cases, the movements are so severe that daily activities like eating, dressing, and attending school become very difficult. Sydenham chorea most commonly affects children between the ages of 5 and 15, and it is more frequent in girls than boys. The condition is closely linked to rheumatic heart disease, so the heart must also be carefully evaluated. Treatment focuses on eliminating the strep infection with antibiotics, managing the involuntary movements with medications, and preventing future strep infections through long-term antibiotic prophylaxis. Most children recover fully within weeks to months, though some may experience relapses. The condition has become less common in developed countries due to better access to antibiotics, but it remains a significant health concern in developing regions.

Key symptoms:

Involuntary jerky movements of the arms, legs, and faceDifficulty with handwriting or drawingSlurred or unclear speechMuscle weaknessTrouble walking or clumsinessFacial grimacingEmotional instability or mood swingsAnxiety or irritabilityDifficulty concentrating or paying attentionTrouble eating or swallowing due to uncontrolled movementsRestlessnessObsessive-compulsive behaviorsMovements that worsen with stress and stop during sleepMilkmaid's grip (inability to maintain a steady hand squeeze)

Clinical phenotype terms (17)— hover any for plain English
Movement abnormality of the tongueHP:0000182Facial grimacingHP:0000273Inappropriate behaviorHP:0000719HemiballismusHP:0100248Septic arthritisHP:0003095Recurrent streptococcus pneumoniae infectionsHP:0005366EndocarditisHP:0100584
Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Sydenham chorea.

View clinical trials →

No actively recruiting trials found for Sydenham chorea at this time.

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

Search ClinicalTrials.gov ↗Join the Sydenham chorea community →

Specialists

19 foundView all specialists →
KP
Kathryn V Roberts, MBBS PhD
Specialist
PI on 1 active trial
HP
Hannah F Jones, MBChB PhD
Specialist
PI on 1 active trial
KC
Kathleen Walker, MB ChB
Specialist
PI on 1 active trial
AV
Allison Vreeland
PALO ALTO, CA
Specialist
2 Sydenham chorea publications
SK
Sonia Khamis
Specialist
1 Sydenham chorea publication
SZ
Sameer M Zuberi
Specialist
1 Sydenham chorea publication
MN
Margherita Nosadini
Specialist
2 Sydenham chorea publications
MM
Michael Morton
Specialist
3 Sydenham chorea publications
RD
Russell C Dale
Specialist
3 Sydenham chorea publications
ME
Michael Eyre
SPANISH FORK, UT
Specialist
2 Sydenham chorea publications
TT
Terrence Thomas
Specialist
2 Sydenham chorea publications
AS
Adrian Sie
Specialist
2 Sydenham chorea publications
TN
Tamsin Newlove-Delgado
Specialist
2 Sydenham chorea publications
CP
Christopher Pittenger
NEW HAVEN, CT
Specialist
2 Sydenham chorea publications
MT
Margo Thienemann
PALO ALTO, CA
Specialist
2 Sydenham chorea publications
JF
Jennifer Frankovich
PALO ALTO, CA
Specialist
2 Sydenham chorea publications
SM
Shekeeb S Mohammad
Specialist
2 Sydenham chorea publications
EF
Emanuela Ferrarin
Specialist
2 Sydenham chorea publications
AM
Armin Raznahan, M.D.
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 Sydenham chorea.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Sydenham chorea

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Caregiver Resources

NORD Caregiver Resources

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

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is my child's chorea, and how long is it expected to last?,Does my child have any signs of rheumatic heart disease, and how will the heart be monitored?,How long will my child need to take preventive antibiotics, and what happens if a dose is missed?,What medications will be used to control the movements, and what are the side effects?,What school accommodations should we request during the active phase?,What are the chances of the chorea coming back, and what can we do to reduce that risk?,Should my child see a psychologist or psychiatrist for the emotional and behavioral changes?

Common questions about Sydenham chorea

What is Sydenham chorea?

Sydenham chorea, also known as St. Vitus' dance or rheumatic chorea, is a neurological disorder that occurs as a complication of rheumatic fever. Rheumatic fever itself is triggered by a throat infection caused by group A streptococcus bacteria (strep throat). After the infection, the body's immune system mistakenly attacks parts of the brain, particularly a region called the basal ganglia, which helps control movement. This leads to the hallmark symptom of chorea — involuntary, jerky, irregular movements that can affect the face, arms, legs, and trunk. Symptoms typically appear weeks to mont

How is Sydenham chorea inherited?

Sydenham chorea follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Sydenham chorea typically begin?

Typical onset of Sydenham chorea is childhood. Age of onset can vary across affected individuals.

Which specialists treat Sydenham chorea?

19 specialists and care centers treating Sydenham chorea are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.