MSeqDR Mitochondrial Disease Portal


 
*:HP: HPO terms, ND: NAMDC terms.
  Most Studied  CPEO, Complex I Deficiency, COXPD1, Leigh, LHON, MELAS, MERRF, NARP, SANDO
Disease Browser
Parent Node:
expandfrontotemporal degeneration with dementia (MONDO:0020137)
Parent Node:
expandfrontotemporal neurodegeneration with movement disorder (MONDO:0017643)
Parent Node:
expandgenetic neurodegenerative disease with dementia (MONDO:0021037)
Parent Node:
expandinherited tremor disorder (MONDO:0017663)
..Starting node
..expandfrontotemporal dementia with motor neuron disease ()

       Child Nodes:
........expandFTDALS ()



 Sister Nodes: 
..expandarthrogryposis ()
..expandataxia with fasciculations ()
..expandataxia-hypogonadism-choroidal dystrophy syndrome ()
..expandautosomal dominant cerebellar ataxia ()
..expandautosomal dominant sensory ataxia 1 ()
..expandautosomal recessive cerebellar ataxia ()
..expandautosomal recessive limb-girdle muscular dystrophy type 2S ()
..expandbehavioral variant of frontotemporal dementia ()
..expandbilateral striopallidodentate calcinosis ()
..expandcataract-ataxia-deafness syndrome ()
..expandcerebellar ataxia-hypogonadism syndrome ()
..expandchildhood-onset benign chorea with striatal involvement ()
..expandchorea ()
..expandchorea, remitting, with nystagmus and cataract ()
..expandchoreoathetosis, familial inverted ()
..expandcorticobasal syndrome ()
..expandepisodic ataxia type 1 ()
..expandETM2 ()
..expandETM3 ()
..expandfamilial infantile bilateral striatal necrosis ()  LSDB  L: 00075;
..expandfrontotemporal dementia with motor neuron disease ()
..expandhereditary geniospasm ()
..expandhereditary hyperekplexia ()
..expandhereditary spastic paraplegia 7 ()  LSDB  L: 00497;
..expandHuntington disease ()
..expandHuntington disease-like 1 ()
..expandHuntington disease-like 3 ()
..expandHuntington disease-like syndrome due to C9ORF72 expansions ()
..expandichthyosis-hepatosplenomegaly-cerebellar degeneration syndrome ()
..expandinherited Creutzfeldt-Jakob disease ()
..expandinherited dystonia ()
..expandjuvenile-onset diabetes mellitus-central and peripheral neurodegeneration syndrome ()
..expandmitochondrial myopathy-cerebellar ataxia-pigmentary retinopathy syndrome ()  LSDB  L: 00532;
..expandmonogenic disease with epilepsy ()
..expandmuscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome ()
..expandmyoclonus-cerebellar ataxia-deafness syndrome ()
..expandneuroacanthocytosis ()
..expandneurodegeneration with brain iron accumulation ()
..expandneuronal intranuclear inclusion disease ()
..expandprimary myoclonus ()
..expandprogressive essential tremor-speech impairment-facial dysmorphism-intellectual disability-abnormal behavior syndrome ()
..expandprogressive non-fluent aphasia ()
..expandprogressive supranuclear palsy ()
..expandrare genetic disease with myoclonus as a major feature ()
..expandRichards-Rundle syndrome ()
..expandsensorineural hearing loss-early graying-essential tremor syndrome ()
..expandspasticity-ataxia-gait anomalies syndrome ()
..expandTourette syndrome ()
..expandtremor, hereditary essential, 1 ()
..expandtremor, hereditary essential, 4 ()
..expandtremor, hereditary essential, 5 ()
..expandWilliams syndrome ()
..expandX-linked cerebellar ataxia ()
   

MONDO is developed by the Monarch Initiative. Human Disease MESH is developed by UMLS.
Further data from MedGen, OMIM,ClinVar, CTD
Term ID:17161
Name:frontotemporal dementia with motor neuron disease
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; see this term). The disease is progressive, with death occurring 2-5 years after onset.
Alternative IDs:
ParentIDs:
TreeNumbers:
Synonyms:frontotemporal dementia with amyotrophic lateral sclerosis; FTD-ALS; FTD-MND
Slim Mappings:
Reference: MedGen:
MeSH:
OMIM:
MSeqDR LSDB:  
Genes:
Phenotypes
Disease Causing ClinVar Variants
MSeqDR Portal