Corticobasal syndrome
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult, Elderly
- Status
- active
A rare neurologic disease characterized by multifaceted motor system dysfunctions and cognitive defects such as asymmetric rigidity, bradykinesia, limb apraxia, and visuospatial dysfunction.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Corticobasal syndrome | |
| fr | Syndrome corticobasal | |
| de | Kortikobasales Syndrom | |
| es | Síndrome corticobasal | |
| it | Sindrome corticobasale | |
| nl | Corticobasaal syndroom | |
| pt | Síndrome corticobasal | |
| pl | Zespół korowo-podstawny | |
| cs | Kortikobazální syndrom | |
| uk | Кортикобазальний синдром | |
| zh | 皮质基底动脉综合征 | |
| tr | Kortikobazal sendrom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G31.0 | NTBT | attributed | validated |
| MONDO | 0018696 | E | validated | |
| MeSH | D000088282 | E | validated | |
| UMLS | C0393570 | 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 (3):
- ORPHA:98535Frontotemporal degeneration with dementia
rare neurological diseases - ORPHA:306708Frontotemporal neurodegeneration with movement disorder
rare genetic diseases; rare neurological diseases - ORPHA:306666Rare parkinsonian syndrome due to neurodegenerative disease
rare neurological diseases
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:278OBSOLETE: Corticobasal degeneration referred to · inactive
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 | Validated | [EXPERT] |
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
22 annotations · validated 2018-12-31| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001336 | Myoclonus | |
| HP:0001300 | Parkinsonism | |
| HP:0007158 | Progressive extrapyramidal muscular rigidity | |
| Frequent (79-30%) | ||
| HP:0002304 | Akinesia | |
| HP:0002067 | Bradykinesia | |
| HP:0000726 | Dementia | |
| HP:0000743 | Frontal release signs | |
| HP:0001288 | Gait disturbance | |
| HP:0004305 | Involuntary movements | |
| HP:0030217 | Limb apraxia | |
| HP:0002451 | Limb dystonia | |
| HP:0045084 | Limb myoclonus | |
| HP:0007301 | Oromotor apraxia | |
| HP:0002172 | Postural instability | |
| HP:0003474 | Somatic sensory dysfunction | |
| HP:0001337 | Tremor | |
| Occasional (29-5%) | ||
| HP:0002381 | Aphasia | |
| HP:0001332 | Dystonia | |
| HP:0002354 | Memory impairment | |
| HP:0011098 | Speech apraxia | |
| Very rare (<4-1%) | ||
| HP:0100022 | Abnormality of movement | |
| HP:0000708 | Atypical behavior | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 30090966[PMID]_23359374[PMID]_29269636[PMID]_21853287[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/454887
- This record as JSON: /data/d/454887.json