Postinfectious autoimmune disease with chorea

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Overview

Postinfectious autoimmune disease with chorea is a condition in which the body's immune system mistakenly attacks parts of the brain after an infection, leading to involuntary, jerky movements called chorea. The most well-known form of this condition is Sydenham chorea (also called St. Vitus' dance), which occurs after a streptococcal (strep) infection, typically strep throat or scarlet fever. In this disease, antibodies that were made to fight the infection cross-react with proteins in a part of the brain called the basal ganglia, which controls movement. This leads to uncontrolled, rapid, irregular movements of the face, arms, legs, and trunk. Patients may also experience emotional changes such as mood swings, anxiety, irritability, and difficulty concentrating. Muscle weakness and trouble with handwriting or fine motor tasks are also common. The condition most often affects children and adolescents, usually appearing weeks to months after the initial infection. Girls are affected more often than boys. In most cases, the chorea improves over time, often within weeks to months, though some patients may have symptoms that last longer or come back. Treatment focuses on managing the involuntary movements, treating any remaining infection, and calming the overactive immune response. Medications such as antibiotics, anti-inflammatory drugs, and drugs that help control movement may be used. With proper treatment, the outlook is generally good, though long-term follow-up is important to prevent recurrences and monitor for related complications such as rheumatic heart disease.

Key symptoms:

Involuntary jerky movements of the arms, legs, and faceDifficulty with handwriting or fine motor tasksMuscle weaknessSlurred or unclear speechTrouble walking or clumsinessMood swings or emotional outburstsAnxiety or irritabilityDifficulty concentrating or paying attentionFacial grimacingRestlessnessDifficulty eating or swallowing in severe casesObsessive-compulsive behaviorsFatigue

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Postinfectious autoimmune disease with chorea.

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No actively recruiting trials found for Postinfectious autoimmune disease with chorea at this time.

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No specialists are currently listed for Postinfectious autoimmune disease with chorea.

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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 Postinfectious autoimmune disease with chorea.

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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.How long is my child likely to have these involuntary movements?,Does my child need long-term antibiotics, and for how long?,Should my child have a heart ultrasound to check for rheumatic heart disease?,What medications can help control the chorea, and what are their side effects?,What should I watch for that would mean I need to bring my child back urgently?,Will my child need school accommodations, and can you provide documentation?,What is the chance that the chorea will come back after it goes away?

Common questions about Postinfectious autoimmune disease with chorea

What is Postinfectious autoimmune disease with chorea?

Postinfectious autoimmune disease with chorea is a condition in which the body's immune system mistakenly attacks parts of the brain after an infection, leading to involuntary, jerky movements called chorea. The most well-known form of this condition is Sydenham chorea (also called St. Vitus' dance), which occurs after a streptococcal (strep) infection, typically strep throat or scarlet fever. In this disease, antibodies that were made to fight the infection cross-react with proteins in a part of the brain called the basal ganglia, which controls movement. This leads to uncontrolled, rapid, ir

How is Postinfectious autoimmune disease with chorea inherited?

Postinfectious autoimmune disease with chorea follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Postinfectious autoimmune disease with chorea typically begin?

Typical onset of Postinfectious autoimmune disease with chorea is childhood. Age of onset can vary across affected individuals.