Frontotemporal dementia

General Information (adopted from Orphanet):

Synonyms, Signs: FTD
Number of Symptoms 18
OrphanetNr: 282
OMIM Id: 172700
600274
600795
607485
ICD-10: G31.0
UMLs: C0338451
MeSH: D057180
MedDRA: 10068968
Snomed: 230270009

Prevalence, inheritance and age of onset:

Prevalence: 3 of 100 000 [Orphanet]
Inheritance: Autosomal dominant
[Orphanet]
Age of onset: Adult
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Frontotemporal degeneration with dementia
 -Rare neurologic disease
Genetic frontotemporal degeneration with dementia
 -Rare genetic disease

Symptom Information: Sort by abundance 

1
 (HPO:0002463) Language impairment 15 / 7739
2
 (HPO:0002145) Frontotemporal dementia 14 / 7739
3
 (HPO:0000737) Irritability 93 / 7739
4
 (HPO:0000748) Inappropriate laughter 5 / 7739
5
 (HPO:0000751) Personality changes 33 / 7739
6
 (HPO:0010529) Echolalia 20 / 7739
7
 (HPO:0000733) Stereotypy 58 / 7739
8
 (HPO:0000741) Apathy 42 / 7739
9
 (HPO:0002591) Polyphagia 25 / 7739
10
 (HPO:0000734) Disinhibition 13 / 7739
11
 (HPO:0002476) Primitive reflex 9 / 7739
12
 (HPO:0000710) Hyperorality 6 / 7739
13
 (HPO:0030213) Emotional blunting 1 / 7739
14
 (HPO:0003745) Sporadic 131 / 7739
15
 (HPO:0002171) Gliosis 48 / 7739
16
 (HPO:0030223) Perseveration 5 / 7739
17
 (HPO:0000006) Autosomal dominant inheritance 2518 / 7739
18
 (HPO:0002529) Neuronal loss in central nervous system 37 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: