Worster-Drought syndrome
Synonyms: Congenital suprabulbar paresis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Autosomal dominant, Not applicable
- Age of onset
- Childhood
- Status
- active
Worster-Drought syndrome (WDS) is a form of cerebral palsy characterized by congenital pseudobulbar (suprabulbar) paresis manifesting as selective weakness of the lips, tongue and soft palate, dysphagia, dysphonia, drooling and jaw jerking.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Worster-Drought syndrome | Congenital suprabulbar paresis |
| fr | Syndrome de Worster-Drought | Parésie suprabulbaire congénitale |
| de | Worster-Drought-Syndrom | Suprabulbäre Parese, kongenitale |
| es | Síndrome de Worster-Drought | Paresia suprabulbar congénita |
| it | Sindrome di Worster-Drought | Paresi soprabulbare congenita |
| nl | Syndroom van Worster-Drought | Congenitale suprabulbaire parese |
| pt | Síndrome Worster-Drought | Parésia suprabulbar congénita |
| pl | Zespół Worstera i Droughta | Wrodzony paraliż podopuszkowy |
| cs | Worster-Droughtův syndrom | Vrozená suprabulbární obrna |
| uk | Синдром Уорстера-Дроута | Вроджений надбульбарний парез |
| zh | Worster-Drought综合征 | 先天性上延髓麻痹 |
| tr | Worster-Drought sendromu | Doğumsal suprabulbar parezi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 185480 | E | validated | |
| ICD-10 | G80.8 | NTBT | attributed | validated |
| ICD-11 | 8D23 | E | specific | validated |
| MONDO | 0008503 | E | validated | |
| MeSH | C536747 | E | validated | |
| UMLS | C0796204 | E | validated | |
| GARD | 5598 | 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:98006Rare neurologic disease
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 | 1-9 / 100 000 | 3.7 / 100 000 | Europe | Value and class | 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
18 annotations · validated 2024-10-15| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001291 | Abnormal cranial nerve morphology | |
| HP:0002167 | Abnormality of speech or vocalization | |
| Frequent (79-30%) | ||
| HP:0100543 | Cognitive impairment | |
| HP:0002307 | Drooling | |
| HP:0002015 | Dysphagia | |
| HP:0001618 | Dysphonia | |
| HP:0002020 | Gastroesophageal reflux | |
| HP:0040262 | Glue ear | |
| HP:0001347 | Hyperreflexia | |
| HP:0033683 | Jaw hyperreflexia | |
| HP:0002205 | Recurrent respiratory infections | |
| HP:0001328 | Specific learning disability | |
| HP:0000183 | Tongue muscle weakness | |
| Occasional (29-5%) | ||
| HP:0007018 | Attention deficit hyperactivity disorder | |
| HP:0000252 | Microcephaly | |
| HP:0001250 | Seizure | |
| HP:0000407 | Sensorineural hearing impairment | |
| HP:0002445 | Tetraplegia | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 30090841[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/3465
- This record as JSON: /data/d/3465.json