loading...| Preferred Name |
Frontotemporal dementia |
| ID |
http://www.orpha.net/ORDO/Orphanet_282 |
| alternative_term |
FTD |
| definition |
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 (see these terms). |
| definition_citation |
Orphanet |
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=282 |
| has_age_of_onset |
Adult |
| has_inheritance |
Autosomal dominant |
| hasDbXref |
ICD-11:6D83 OMIM:600795 MedDRA:10068968 OMIM:607485 OMIM:600274 MeSH:D057180 UMLS:C0338451 OMIM:172700 |
| label |
Frontotemporal dementia |
| notation |
Clinical group ORPHA:282 |
| prefixIRI |
ORDO:Orphanet_282 |
| prefLabel |
Frontotemporal dementia |
| present_in |
Italy AND has_annual_incidence_average_value : 3.05 AND has_annual_incidence_range : 1-9 / 100 000 Europe AND has_point_prevalence_average_value : 3.0 AND has_point_prevalence_range : 1-9 / 100 000 |
| subClassOf |
http://www.orpha.net/ORDO/Orphanet_276061 |