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Disease Profile

Sydenham’s chorea

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Childhood

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ICD-10

I02.0 I02.9

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

St. Vitus dance; Sydenham chorea; Rheumatic chorea

Summary

Sydenham's chorea is a neurological disorder characterized by rapid, jerky, irregular, and involuntary movements (chorea), especially of the face and limbs.[1][2] Additional symptoms may include muscle weakness, slurred speech, headaches, and seizures. Children with Sydenham's chorea often have emotional or behavioral problems such as obsessive-compulsive disorder, distractibility, irritability, and inappropriate outbursts of laughing or crying.[2] Sydenham's chorea mostly affects children and adolescents and usually follows a Streptococcal infection by anywhere form 1-8 months.[1][2] Sydenham's chorea is one of the major clinical signs of acute rheumatic fever.[2] The uncontrolled movements are often worse during periods of stress, fatigue, or excitement. In some cases, only one side of the body is affected. Sydenham's chorea usually resolves within 3 weeks to 3 months. However, symptoms may last longer in some cases.[1]

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
30%-79% of people have these symptoms
Chorea
0002072
Dysarthria
Difficulty articulating speech
0001260
Emotional lability
Emotional instability
0000712
Facial grimacing
0000273
Generalized hypotonia
Decreased muscle tone
Low muscle tone

[ more ]

0001290
Headache
Headaches
0002315
Hemiballismus
0100248
Inappropriate behavior
0000719
Irritability
Irritable
0000737
Movement abnormality of the tongue
0000182
Obsessive-compulsive behavior
Obsessive compulsive behavior
0000722
Unsteady gait
Unsteady walk
0002317
5%-29% of people have these symptoms
Endocarditis
0100584
Erythema
0010783
Recurrent streptococcus pneumoniae infections
0005366
Septic arthritis
0003095

Treatment

In most cases, patients with Sydenham's chorea recover fully with no treatment. Drugs that have been used to treat patients with significant movement problems include corticosteroids, valproic acid, diazepam, chlorpromazine, and carbamazepine. In patients who do not respond to these drugs, haloperidol or pimozide may be used. In addition, patients with Sydenham's chorea are usually treated with antibiotics to prevent another Streptococcal infection and to minimize the risk of rheumatic heart disease. Sydenham's chorea usually resolves within 12-15 months, though symptoms may persist for two years or more. Up to 30% of patients experience a recurrence of Sydenham's chorea within a few years. Some believe that treatment with an antibiotic reduces the risk of recurrence.[2]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
    • The National Institute of Neurological Disorders and Stroke (NINDS) (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
    • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

      In-Depth Information

      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • Orphanet is a European database for information on rare diseases and orphan drugs.
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Sydenham's chorea. Click on the link to view a sample search on this topic.

        References

        1. Okun, Michael. Sydenham Chorea. National Organization for Rare Disorders (NORD). 2010; https://rarediseases.org/rare-diseases/sydenham-chorea/.
        2. Cruse, Robert. Sydenham chorea. UpToDate. April 29, 2014; https://www.uptodate.com/contents/sydenham-chorea.

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