Sydenham chorea
- Type
- Particular clinical situation in a disease or syndrome
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare acquired autoimmune movement disorder characterized by chorea, hypotonia, and emotional lability. Additional clinical features may include motor impersistence and various neuropsychiatric symptoms. As a major clinical manifestation of acute rheumatic fever (ARF), the disorder typically emerges as a post-infectious complication of a group A streptococcal infection and frequently presents alongside cardiac involvement, such as clinical or subclinical carditis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Sydenham chorea | |
| fr | Chorée de Sydenham | |
| de | Sydenham-Chorea | |
| es | Corea de Sydenham | |
| it | Corea di Sydenham | |
| nl | Sydenhamchorea | |
| pt | Coreia de Sydenham | |
| pl | pląsawica Sydenhama | |
| cs | Sydenhamova chorea | |
| uk | Хорея Сиденхема | |
| zh | Sydenham舞蹈病 | |
| tr | Sydenham koresi |
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I02.0 | BTNT | attributed | validated |
| ICD-10 | I02.9 | BTNT | attributed | validated |
| ICD-11 | 1B42 | NTBT | index | validated |
| MONDO | 0017648 | E | validated | |
| UMLS | C0152113 | E | validated | |
| GARD | 7716 | E | validated | |
| MedDRA | 10042732 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:306727Postinfectious autoimmune disease with chorea
rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
17 annotations · validated 2018-04-18| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0000708 | Atypical behavior | |
| HP:0002072 | Chorea | |
| HP:0000722 | Compulsive behaviors | |
| HP:0001260 | Dysarthria | |
| HP:0000712 | Emotional lability | |
| HP:0000273 | Facial grimacing | |
| HP:0001290 | Generalized hypotonia | |
| HP:0002315 | Headache | |
| HP:0100248 | Hemiballismus | |
| HP:0000719 | Inappropriate behavior | |
| HP:0000737 | Irritability | |
| HP:0000182 | Movement abnormality of the tongue | |
| HP:0002317 | Unsteady gait | |
| Occasional (29-5%) | ||
| HP:0100584 | Endocarditis | |
| HP:0010783 | Erythema | |
| HP:0005366 | Recurrent streptococcus pneumoniae infections | |
| HP:0003095 | Septic arthritis | |
Annotation source: 21386976[PMID]_16533893[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/306731
- This record as JSON: /data/d/306731.json