MCID: SYD002
MIFTS: 34

Sydenham Chorea

Categories: Cardiovascular diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Sydenham Chorea

MalaCards integrated aliases for Sydenham Chorea:

Name: Sydenham Chorea 40 20 53 58 36
Rheumatic Chorea 20 70 32
Saint Vitus Dance 40 53
Sydenham's Chorea 73 20
St. Vitus Dance 20

Classifications:

Orphanet: 58  
Rare neurological diseases


Summaries for Sydenham Chorea

NINDS : 53 Sydenham chorea (SC) is a neurological disorder of childhood resulting from infection via Group A beta-hemolytic streptococcus (GABHS), the bacterium that causes rheumatic fever.  SC is characterized by rapid, irregular, and aimless involuntary movements of the arms and legs, trunk, and facial muscles.  It affects girls more often than boys and typically occurs between 5 and 15 years of age. Some children will have a sore throat several weeks before the symptoms begin, but the disorder can also strike up to 6 months after the fever or infection has cleared.  Symptoms can appear gradually or all at once, and also may include uncoordinated movements, muscular weakness, stumbling and falling, slurred speech, difficulty concentrating and writing, and emotional instability.  The symptoms of SC can vary from a halting gait and slight grimacing to involuntary movements that are frequent and severe enough to be incapacitating.  The random, writhing movements of chorea are caused by an auto-immune reaction to the bacterium that interferes with the normal function of a part of the brain (the basal ganglia) that controls motor movements.  Due to better sanitary conditions and the use of antibiotics to treat streptococcal infections, rheumatic fever, and consequently SC, are rare in North America and Europe.  The disease can still be found in developing nations.

MalaCards based summary : Sydenham Chorea, also known as rheumatic chorea, is related to chorea gravidarum and rheumatic encephalitis. The drugs Haloperidol and Penicillin V have been mentioned in the context of this disorder. Affiliated tissues include heart, tongue and brain, and related phenotypes are emotional lability and dysarthria

GARD : 20 Sydenham's chorea is a neurological disorder characterized by rapid, jerky, irregular, and involuntary movements (chorea), especially of the face and limbs. 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. Sydenham's chorea mostly affects children and adolescents and usually follows a Streptococcal infection by anywhere form 1-8 months. Sydenham's chorea is one of the major clinical signs of acute rheumatic fever. 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.

KEGG : 36 Sydenham chorea is a disabling pediatric hyperkinetic and neuropsychiatric disorder following streptococcal infection. Sydenham chorea occurs in approximately 10% of acute rheumatic fever and is one of its major manifestations. The disease may last for weeks or months, with a high risk of recurrence. The common features of rheumatic fever include sore throats due to streptococcal infections, rashes, pains in the limbs, and subcutaneous nodules, and this form of chorea typically presents, after varying intervals, with twitching of the mouth and limbs, shrugging of the shoulders, and clumsy gait. Those with this form of chorea are less likely to have severe cardiac or rheumatologic complications. The pathogenesis has been considered to be an autoantibody-mediated basal ganglia dysfunction. Anti-inflammatory treatments such as steroids, plasmapheresis and intravenous immunoglobulin treatment are effective.

Wikipedia : 73 Sydenham's chorea, also known as chorea minor and historically and occasionally referred to as St Vitus'... more...

Related Diseases for Sydenham Chorea

Diseases related to Sydenham Chorea via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 92)
# Related Disease Score Top Affiliating Genes
1 chorea gravidarum 11.2
2 rheumatic encephalitis 11.2
3 chorea, childhood-onset, with psychomotor retardation 10.9
4 choreatic disease 10.9
5 rheumatic fever-related antigen 10.7
6 rheumatic fever 10.7
7 obsessive-compulsive disorder 10.5
8 movement disease 10.3
9 tic disorder 10.3
10 dystonia 10.3
11 gilles de la tourette syndrome 10.2
12 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
13 encephalitis 10.2
14 pseudobulbar affect 10.2
15 hypotonia 10.2
16 moyamoya disease 1 10.2
17 heart disease 10.2
18 enthesopathy 10.2
19 vasculitis 10.2
20 seizure disorder 10.2
21 tremor 10.2
22 autoimmune disease 10.0
23 hashimoto thyroiditis 10.0
24 huntington disease 10.0
25 attention deficit-hyperactivity disorder 10.0
26 pulmonary hypertension 10.0
27 dyskinetic cerebral palsy 10.0
28 rheumatic heart disease 10.0
29 parkinsonism 10.0
30 henoch-schoenlein purpura 10.0
31 hemopericardium 10.0
32 mitral valve insufficiency 10.0
33 pericardial effusion 10.0
34 conn's syndrome 10.0
35 mitral valve stenosis 10.0
36 cerebral palsy 10.0
37 pharyngitis 10.0
38 antiphospholipid syndrome 10.0
39 purpura 10.0
40 von economo's disease 10.0
41 thyroiditis 10.0
42 arthritis 10.0
43 lingual-facial-buccal dyskinesia 10.0
44 streptococcal group a invasive disease 10.0
45 paroxysmal choreoathetosis 10.0
46 hand skill, relative 10.0
47 systemic lupus erythematosus 10.0
48 medulloblastoma 10.0
49 migraine with or without aura 1 10.0
50 schizophrenia 10.0

Graphical network of the top 20 diseases related to Sydenham Chorea:



Diseases related to Sydenham Chorea

Symptoms & Phenotypes for Sydenham Chorea

Human phenotypes related to Sydenham Chorea:

58 31 (show all 17)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 emotional lability 58 31 frequent (33%) Frequent (79-30%) HP:0000712
2 dysarthria 58 31 frequent (33%) Frequent (79-30%) HP:0001260
3 chorea 58 31 frequent (33%) Frequent (79-30%) HP:0002072
4 irritability 58 31 frequent (33%) Frequent (79-30%) HP:0000737
5 obsessive-compulsive behavior 58 31 frequent (33%) Frequent (79-30%) HP:0000722
6 hemiballismus 58 31 frequent (33%) Frequent (79-30%) HP:0100248
7 headache 58 31 frequent (33%) Frequent (79-30%) HP:0002315
8 generalized hypotonia 58 31 frequent (33%) Frequent (79-30%) HP:0001290
9 unsteady gait 58 31 frequent (33%) Frequent (79-30%) HP:0002317
10 facial grimacing 58 31 frequent (33%) Frequent (79-30%) HP:0000273
11 movement abnormality of the tongue 58 31 frequent (33%) Frequent (79-30%) HP:0000182
12 inappropriate behavior 58 31 frequent (33%) Frequent (79-30%) HP:0000719
13 erythema 58 31 occasional (7.5%) Occasional (29-5%) HP:0010783
14 endocarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100584
15 septic arthritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0003095
16 recurrent streptococcus pneumoniae infections 58 31 occasional (7.5%) Occasional (29-5%) HP:0005366
17 behavioral abnormality 58 Frequent (79-30%)

Drugs & Therapeutics for Sydenham Chorea

Drugs for Sydenham Chorea (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 22)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Haloperidol Approved 52-86-8 3559
2
Penicillin V Approved, Vet_approved 87-08-1 6869
3
Dopamine Approved 62-31-7, 51-61-6 681
4 Neurotransmitter Agents
5 Haloperidol decanoate
6 Psychotropic Drugs
7 Gastrointestinal Agents
8 Immunoglobulins, Intravenous
9 Anti-Infective Agents
10 Antiemetics
11 Dopamine Agents
12 gamma-Globulins
13 Anti-Bacterial Agents
14 Rho(D) Immune Globulin
15 Antipsychotic Agents
16 Dopamine Antagonists
17 penicillins
18 Immunoglobulins
19 Antibodies
20 Immunologic Factors
21 Dopamine agonists
22 Autoantibodies

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Intravenous Immunoglobulins as Effective Treatment in Sydenham's Chorea Completed NCT00615797 standard interventions penicillin VK and haloperidol
2 Sydenham's Chorea: Is There a Link Between Neuropsychiatric Symptoms and Anti-dopamine Receptor Autoantibodies? Enrolling by invitation NCT04084977

Search NIH Clinical Center for Sydenham Chorea

Genetic Tests for Sydenham Chorea

Anatomical Context for Sydenham Chorea

MalaCards organs/tissues related to Sydenham Chorea:

40
Heart, Tongue, Brain

Publications for Sydenham Chorea

Articles related to Sydenham Chorea:

(show top 50) (show all 180)
# Title Authors PMID Year
1
A sydenham chorea attack associated with COVID-19 infection. 61
33615279 2021
2
Acute chorea: case series from the emergency room of a Brazilian tertiary-level center. 61
33729326 2021
3
Sydenham Chorea: Putaminal Enlargement. 61
32851928 2021
4
Neurologic complications of rheumatic fever. 61
33632442 2021
5
[Bertolt Brecht. Did he really have rheumatic fever?] 61
32761253 2020
6
Efficacy of levetiracetam in the treatment of Sydenham chorea. 61
32445412 2020
7
Chorea in children: etiology, diagnostic approach and management. 61
32776155 2020
8
Sydenham chorea in a12- year old Saudi girl. 61
32566771 2020
9
Preliminary data on prednisone effectiveness in children with Sydenham chorea. 61
31965299 2020
10
Serum periostin levels in acute rheumatic fever: is it useful as a new biomarker? 61
31648625 2020
11
Levodopa-Responsive Chorea: A Review. 61
32189864 2020
12
Autoantibody Biomarkers for Basal Ganglia Encephalitis in Sydenham Chorea and Pediatric Autoimmune Neuropsychiatric Disorder Associated With Streptococcal Infections. 61
32670106 2020
13
Simultaneous Occurrence of Sydenham Chorea with Erythema Marginatum: A Case of Rheumatic Fever. 61
32110138 2020
14
[CHOREA GRAVIDARUM: CASE REPORT AND REVIEW OF THE LITERATURE]. 61
31738489 2019
15
Rheumatic Chorea as the First Presenting Sign in a 13-year-old Female Child. 61
31637146 2019
16
Chorea. 61
31356291 2019
17
Molecular Mimicry, Autoimmunity, and Infection: The Cross-Reactive Antigens of Group A Streptococci and their Sequelae. 61
31373269 2019
18
Focus on Cardiologic Findings in 30 Children With PANS/PANDAS: An Italian Single-Center Observational Study. 61
31632938 2019
19
Longitudinal outcomes of children with pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS). 61
29119300 2018
20
Rare case of chorea-hyperglycaemia-basal ganglia (C-H-BG) syndrome. 61
29666090 2018
21
Principles and approaches to the treatment of immune-mediated movement disorders. 61
29289523 2018
22
[A boy with involuntary movements]. 61
29350121 2018
23
A retrospective study: Acute rheumatic fever and post-streptococcal reactive arthritis in Japan. 61
28442182 2017
24
Clinical Value of Perfusion Abnormalities of Brain on Technetium-99m HMPAO Single-Photon Emission Computed Tomography in Children With Sydenham Chorea. 61
27920268 2017
25
Systematic review of immunoglobulin use in paediatric neurological and neurodevelopmental disorders. 61
27900773 2017
26
Treatment of Sydenham's Chorea: A Review of the Current Evidence. 61
28589057 2017
27
Acute rheumatic fever outbreak in southern central European country. 61
27815733 2017
28
Autoimmunity against dopamine receptors in neuropsychiatric and movement disorders: a review of Sydenham chorea and beyond. 61
26454143 2016
29
Language Impairment in Adolescents With Sydenham Chorea. 61
26296451 2015
30
Language Impairment in Adolescents with Sydenham Chorea. 61
26933544 2015
31
Autoantibodies in movement and psychiatric disorders: updated concepts in detection methods, pathogenicity, and CNS entry. 61
26083906 2015
32
A New Alternative Drug With Fewer Adverse Effects in the Treatment of Sydenham Chorea: Levetiracetam Efficacy in a Child. 61
26166232 2015
33
Sydenham Chorea and PANDAS in South Africa: Review of Evidence and Recommendations for Management in Resource-Poor Countries. 61
25227516 2015
34
Therapeutic plasma apheresis as a treatment for 35 severely ill children and adolescents with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. 61
25658452 2015
35
Antineuronal antibodies in a heterogeneous group of youth and young adults with tics and obsessive-compulsive disorder. 61
25658702 2015
36
Sydenham chorea in a 5-year-old Saudi patient. 61
25630786 2015
37
Sydenham chorea in a 5-year-old Saudi patient. 61
25630787 2015
38
The usefulness of immunotherapy in pediatric neurodegenerative disorders: A systematic review of literature data. 61
26266339 2015
39
Sydenham Chorea: rare consequence of rheumatic fever. 61
25493130 2014
40
Benign hereditary chorea: a case report and brief review of inherited choreas. 61
25108317 2014
41
Anti-basal ganglia antibodies in primary obsessive-compulsive disorder: systematic review and meta-analysis. 61
24986387 2014
42
Rheumatic fever, autoimmunity, and molecular mimicry: the streptococcal connection. 61
24892819 2014
43
Sydenham chorea in a 5-year-old Saudi patient. 61
24983288 2014
44
Acute-onset choreiform movements in a previously healthy 4-year-old patient. 61
24378857 2014
45
Brain human monoclonal autoantibody from sydenham chorea targets dopaminergic neurons in transgenic mice and signals dopamine D2 receptor: implications in human disease. 61
24184556 2013
46
Autoantibody-associated movement disorders. 61
24203856 2013
47
Narcolepsy-cataplexy: is streptococcal infection a trigger? 61
23493659 2013
48
Dopamine receptor autoantibodies correlate with symptoms in Sydenham's chorea. 61
24073196 2013
49
[Antiphospolipid syndrome related chorea gravidarum case with psychotic symptoms misdiagnosed as conversion disorder: case report]. 61
24310096 2013
50
A national prospective surveillance study of acute rheumatic fever in Australian children. 61
22926211 2013

Variations for Sydenham Chorea

Expression for Sydenham Chorea

Search GEO for disease gene expression data for Sydenham Chorea.

Pathways for Sydenham Chorea

GO Terms for Sydenham Chorea

Sources for Sydenham Chorea

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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