Preferred Name |
Frontotemporal dementia |
|
Synonyms |
FTD |
|
Definitions |
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). |
|
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 OMIM:172700 OMIM:607485 UMLS:C0338451 MeSH:D057180 OMIM:600274 MedDRA:10068968 |
|
label |
Frontotemporal dementia |
|
notation |
Clinical group ORPHA:282 |
|
prefixIRI |
ORDO:Orphanet_282 |
|
prefLabel |
Frontotemporal dementia |
|
present_in |
Europe AND has_point_prevalence_average_value : 3.0 AND has_point_prevalence_range : 1-9 / 100 000 Italy AND has_annual_incidence_average_value : 3.05 AND has_annual_incidence_range : 1-9 / 100 000 |
|
subClassOf |
http://www.orpha.net/ORDO/Orphanet_98535 |