MCID: SYD002
MIFTS: 45

Sydenham Chorea

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Sydenham Chorea

MalaCards integrated aliases for Sydenham Chorea:

Name: Sydenham Chorea 41 53 54 59
Sydenham's Chorea 76 53 37
Saint Vitus Dance 41 54
Rheumatic Chorea 73
St. Vitus Dance 53

Classifications:

Orphanet: 59  
Rare neurological diseases


Summaries for Sydenham Chorea

NINDS : 54 Sydenham chorea (SD) is a neurological disorder of childhood resulting from infection via Group A beta-hemolytic streptococcus (GABHS), the bacterium that causes rheumatic fever.  SD 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 SD 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 SD, are rare in North America and Europe.  The disease can still be found in developing nations. 

MalaCards based summary : Sydenham Chorea, also known as sydenham's chorea, is related to rheumatic encephalitis and neuronitis. An important gene associated with Sydenham Chorea is BDNF (Brain Derived Neurotrophic Factor), and among its related pathways/superpathways are PEDF Induced Signaling and Akt Signaling. The drugs Anti-Bacterial Agents and Anti-Infective Agents have been mentioned in the context of this disorder. Affiliated tissues include brain, tongue and heart, and related phenotypes are movement abnormality of the tongue and facial grimacing

NIH Rare Diseases : 53 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.

Wikipedia : 76 Sydenham\'s chorea (SC) or chorea minor (historically and traditionally referred to as St Vitus\' dance)... more...

Related Diseases for Sydenham Chorea

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:

59 32 (show all 17)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 movement abnormality of the tongue 59 32 frequent (33%) Frequent (79-30%) HP:0000182
2 facial grimacing 59 32 frequent (33%) Frequent (79-30%) HP:0000273
3 emotional lability 59 32 frequent (33%) Frequent (79-30%) HP:0000712
4 inappropriate behavior 59 32 frequent (33%) Frequent (79-30%) HP:0000719
5 obsessive-compulsive behavior 59 32 frequent (33%) Frequent (79-30%) HP:0000722
6 irritability 59 32 frequent (33%) Frequent (79-30%) HP:0000737
7 dysarthria 59 32 frequent (33%) Frequent (79-30%) HP:0001260
8 generalized hypotonia 59 32 frequent (33%) Frequent (79-30%) HP:0001290
9 chorea 59 32 frequent (33%) Frequent (79-30%) HP:0002072
10 headache 59 32 frequent (33%) Frequent (79-30%) HP:0002315
11 unsteady gait 59 32 frequent (33%) Frequent (79-30%) HP:0002317
12 hemiballismus 59 32 frequent (33%) Frequent (79-30%) HP:0100248
13 septic arthritis 59 32 occasional (7.5%) Occasional (29-5%) HP:0003095
14 recurrent streptococcus pneumoniae infections 59 32 occasional (7.5%) Occasional (29-5%) HP:0005366
15 erythema 59 32 occasional (7.5%) Occasional (29-5%) HP:0010783
16 endocarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100584
17 behavioral abnormality 59 Frequent (79-30%)

Drugs & Therapeutics for Sydenham Chorea

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

(show all 27)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Anti-Bacterial Agents Phase 2,Not Applicable
2 Anti-Infective Agents Phase 2,Not Applicable
3 penicillins Phase 2,Not Applicable
4
Dopamine Approved Not Applicable 51-61-6, 62-31-7 681
5
Haloperidol Approved Not Applicable 52-86-8 3559
6
Penicillin V Approved, Vet_approved Not Applicable 87-08-1 6869
7
Epinephrine Approved, Vet_approved 51-43-4 5816
8
Racepinephrine Approved 329-65-7 838
9 Antibodies Not Applicable
10 Antiemetics Not Applicable
11 Antipsychotic Agents Not Applicable
12 Autonomic Agents Not Applicable
13 Central Nervous System Depressants Not Applicable
14 decanoic acid Not Applicable
15 Dopamine Agents Not Applicable
16 Dopamine Antagonists Not Applicable
17 gamma-Globulins Not Applicable
18 Gastrointestinal Agents Not Applicable
19 Haloperidol decanoate Not Applicable
20 Immunoglobulins Not Applicable
21 Immunoglobulins, Intravenous Not Applicable
22 Neurotransmitter Agents Not Applicable
23 Peripheral Nervous System Agents Not Applicable
24 Psychotropic Drugs Not Applicable
25 Rho(D) Immune Globulin Not Applicable
26 Tranquilizing Agents Not Applicable
27 Epinephryl borate

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Preventive Measures for Childhood-Onset Obsessive-Compulsive Disorder and Tic Disorders (PANDAS Subgroup) Completed NCT00001359 Phase 2 Penicillin or Placebo
2 Intravenous Immunoglobulins as Effective Treatment in Sydenham's Chorea Completed NCT00615797 Not Applicable standard interventions penicillin VK and haloperidol
3 Brain Imaging of Childhood Onset Psychiatric Disorders, Endocrine Disorders and Healthy Volunteers Recruiting NCT00001246

Search NIH Clinical Center for Sydenham Chorea

Genetic Tests for Sydenham Chorea

Anatomical Context for Sydenham Chorea

MalaCards organs/tissues related to Sydenham Chorea:

41
Brain, Tongue, Heart, Thyroid

Publications for Sydenham Chorea

Articles related to Sydenham Chorea:

(show all 36)
# Title Authors Year
1
Clinical Value of Perfusion Abnormalities of Brain on Technetium-99m HMPAO Single-Photon Emission Computed Tomography in Children With Sydenham Chorea. ( 27920268 )
2016
2
Autoimmunity against dopamine receptors in neuropsychiatric and movement disorders: a review of Sydenham chorea and beyond. ( 26454143 )
2016
3
A New Alternative Drug With Fewer Adverse Effects in the Treatment of Sydenham Chorea: Levetiracetam Efficacy in a Child. ( 26166232 )
2015
4
Language Impairment in Adolescents With Sydenham Chorea. ( 26296451 )
2015
5
Sydenham chorea in a 5-year-old Saudi patient. ( 25630786 )
2015
6
Sydenham chorea in a 5-year-old Saudi patient. ( 25630787 )
2015
7
Language Impairment in Adolescents with Sydenham Chorea. ( 26933544 )
2015
8
Sydenham Chorea and PANDAS in South Africa: Review of Evidence and Recommendations for Management in Resource-Poor Countries. ( 25227516 )
2014
9
Sydenham chorea in a 5-year-old Saudi patient. ( 24983288 )
2014
10
Sydenham Chorea: rare consequence of rheumatic fever. ( 25493130 )
2014
11
Immune-mediated extrapyramidal movement disorders, including Sydenham chorea. ( 23622334 )
2013
12
Brain human monoclonal autoantibody from sydenham chorea targets dopaminergic neurons in transgenic mice and signals dopamine D2 receptor: implications in human disease. ( 24184556 )
2013
13
Striatal microinjection of Sydenham chorea antibodies: using a rat model to examine the dopamine hypothesis. ( 21647711 )
2012
14
Magnetic resonance imaging in Sydenham chorea. ( 22539244 )
2012
15
Behavioral, pharmacological, and immunological abnormalities after streptococcal exposure: a novel rat model of Sydenham chorea and related neuropsychiatric disorders. ( 22534626 )
2012
16
Treatment of sydenham chorea with intravenous immunoglobulin. ( 21868369 )
2012
17
Antibody binding to neuronal surface in Sydenham chorea, but not in PANDAS or Tourette syndrome. ( 21411742 )
2011
18
The Wilson films--Sydenham chorea. ( 22170274 )
2011
19
Reversible striatal hypermetabolism in a case of rare adult-onset Sydenham chorea on two sequential 18F-FDG PET studies. ( 21215457 )
2011
20
Beneficial use of immunoglobulins in the treatment of Sydenham chorea. ( 20349351 )
2010
21
Sydenham chorea and Hashimoto thyroiditis: an unusual association. ( 20350965 )
2010
22
Hypermetabolism in bilateral basal ganglia in Sydenham chorea on F-18 FDG PET-CT. ( 19352269 )
2009
23
Increased anti-saccade latency is an isolated lingering abnormality in Sydenham chorea. ( 19491640 )
2009
24
Remission of concomitant Henoch-SchAPenlein purpura and Sydenham chorea after intravenous corticosteroids. ( 19305078 )
2009
25
Neurologic and cardiac findings in children with Sydenham chorea. ( 17352948 )
2007
26
Sydenham chorea: a case report and review of the literature. ( 17228216 )
2007
27
[A case of adult-onset Sydenham chorea accompanied with psychiatric symptoms]. ( 16519113 )
2006
28
Autoimmune neuropsychiatric disorders associated with streptococcal infection: Sydenham chorea, PANDAS, and PANDAS variants. ( 16970875 )
2006
29
Obsessive-compulsive symptoms among patients with Sydenham chorea. ( 15860349 )
2005
30
Obsessive compulsive behavior, hyperactivity, and attention deficit disorder in Sydenham chorea. ( 15911817 )
2005
31
Recurrence of Sydenham chorea: implications for pathogenesis. ( 15313844 )
2004
32
Treatment of Sydenham chorea with corticosteroids. ( 14639684 )
2003
33
Clinical, laboratory, psychiatric and magnetic resonance findings in patients with Sydenham chorea. ( 12811441 )
2003
34
Mimicry and autoantibody-mediated neuronal cell signaling in Sydenham chorea. ( 12819778 )
2003
35
Sydenham Chorea in Children. ( 12791195 )
2003
36
Anti-basal ganglia antibody abnormalities in Sydenham chorea. ( 12620655 )
2003

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

Cellular components related to Sydenham Chorea according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 8.96 CXCL10 CXCL9
2 extracellular space GO:0005615 8.8 BDNF CXCL10 CXCL9

Biological processes related to Sydenham Chorea according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 regulation of signaling receptor activity GO:0010469 9.58 BDNF CXCL10 CXCL9
2 inflammatory response GO:0006954 9.54 CXCL10 CXCL9
3 regulation of cell proliferation GO:0042127 9.52 CXCL10 CXCL9
4 response to lipopolysaccharide GO:0032496 9.51 CXCL10 CXCL9
5 defense response to virus GO:0051607 9.49 CXCL10 CXCL9
6 chemotaxis GO:0006935 9.48 CXCL10 CXCL9
7 defense response GO:0006952 9.46 CXCL10 CXCL9
8 chemokine-mediated signaling pathway GO:0070098 9.43 CXCL10 CXCL9
9 cell-cell signaling GO:0007267 9.43 BDNF CXCL10 CXCL9
10 positive regulation of release of sequestered calcium ion into cytosol GO:0051281 9.37 CXCL10 CXCL9
11 positive regulation of leukocyte chemotaxis GO:0002690 9.32 CXCL10 CXCL9
12 positive regulation of cAMP-mediated signaling GO:0043950 9.16 CXCL10 CXCL9
13 positive regulation of cAMP metabolic process GO:0030816 8.96 CXCL10 CXCL9
14 T cell chemotaxis GO:0010818 8.62 CXCL10 CXCL9

Molecular functions related to Sydenham Chorea according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.26 CXCL10 CXCL9
2 chemokine activity GO:0008009 9.16 CXCL10 CXCL9
3 CXCR chemokine receptor binding GO:0045236 8.96 CXCL10 CXCL9
4 CXCR3 chemokine receptor binding GO:0048248 8.62 CXCL10 CXCL9

Sources for Sydenham Chorea

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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