Frontotemporal dementia
Synonyms: FTD
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Autosomal dominant
- Age of onset
- Adult
- Status
- active
Frontotemporal dementia (FTD) comprises a group of neurodegenerative disorders, characterized by progressive changes in behavior, executive dysfunction and language impairment, as a result of degeneration of the medial prefrontal and frontoinsular cortices. Four clinical subtypes have been identified: semantic dementia, progressive non-fluent aphasia, behavioral variant FTD and right temporal lobar atrophy.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Frontotemporal dementia | FTD |
| fr | Démence fronto-temporale | DFT · FTD |
| de | Frontotemporale Demenz | = en |
| es | Demencia frontotemporal | DFT |
| it | Demenza frontotemporale | = en |
| nl | Frontotemporale dementie | = en |
| pt | Demência fronto-temporal | |
| pl | Demencja czołowo-skroniowa | = en |
| cs | Frontotemporální demence | = en |
| uk | Лобно-скронева деменція | = en |
| zh | 额颞叶痴呆 | = en |
| tr | Frontotemporal demans | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 172700 | BTNT | validated | |
| OMIM | 600274 | E | validated | |
| OMIM | 600795 | BTNT | validated | |
| OMIM | 607485 | BTNT | validated | |
| ICD-11 | 6D83 | E | specific | validated |
| MONDO | 0017276 | E | validated | |
| MONDO | 17276 | E | validated | |
| MeSH | D057180 | E | validated | |
| UMLS | C0338451 | E | validated | |
| GARD | 8436 | E | validated | |
| MedDRA | 10068968 | 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 (2):
- ORPHA:98535Frontotemporal degeneration with dementia
rare neurological diseases - ORPHA:276061Genetic frontotemporal degeneration with dementia
rare genetic diseases
Codes that Orphanet moved or referred to this one:
- ORPHA:293848Frontotemporal dementia, right temporal atrophy variant moved to · inactive
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 3 / 100 000 | Europe | Value and class | Not yet validated | 20971753[PMID] |
| Annual incidence | 1-9 / 100 000 | 3.05 / 100 000 | Italy | Value and class | Validated | 30979859[PMID] |
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/282
- This record as JSON: /data/d/282.json