JSON

frontotemporal dementia with motor neuron disease

^ http://purl.obolibrary.org/obo/MONDO_0017161


Frontotemporal dementia with motor neuron disease (FTD-MND) is a type of frontotemporal lobar degeneration characterized by the insidious onset (between the ages of 38-78 years) of dementia-associated psychiatric symptoms (e.g. personality changes, uninhibited behavior, irritability, aggressiveness), memory difficulties, global intellectual impairment, emotional disorders and transcortical motor aphasia that eventually leads to mutism, in addition to the manifestations of motor neuron disease such as neurogenic muscular wasting (similar to what is seen in amyotrophic lateral sclerosis). The disease is progressive, with death occurring 2-5 years after onset. [ Orphanet:275872 ]

Term info

database cross reference
  • OMIMPS:105550 (MONDO:equivalentTo)
  • UMLS:CN239493 (MONDO:equivalentTo)
  • MESH:C566288 (MONDO:equivalentTo)
  • Orphanet:275872 (MONDO:equivalentTo)
Subsets

ordo_disease

abbreviation
FTDALS [ MONDO:0000712 ]

definition

Frontotemporal dementia with motor neuron disease (FTD-MND) is a type of frontotemporal lobar degeneration characterized by the insidious onset (between the ages of 38-78 years) of dementia-associated psychiatric symptoms (e.g. personality changes, uninhibited behavior, irritability, aggressiveness), memory difficulties, global intellectual impairment, emotional disorders and transcortical motor aphasia that eventually leads to mutism, in addition to the manifestations of motor neuron disease such as neurogenic muscular wasting (similar to what is seen in amyotrophic lateral sclerosis). The disease is progressive, with death occurring 2-5 years after onset.

exactMatch

https://omim.org/phenotypicSeries/PS105550, http://purl.obolibrary.org/obo/Orphanet_275872, http://identifiers.org/mesh/C566288, http://linkedlifedata.com/resource/umls/id/CN239493

has exact synonym

FTDALS, FTD-MND, frontotemporal dementia with amyotrophic lateral sclerosis, frontotemporal dementia with ALS, FTD-ALS

id

MONDO:0017161