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Frontotemporal dementia with motor neuron disease

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.

ORPHA:275872

Classification level: Disorder
  • Synonym(s):
    • FTD-ALS
    • FTD-MND
    • Frontotemporal dementia with amyotrophic lateral sclerosis
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: G31.0
  • OMIM: 105550  608030  612069  613954  615911  616437  616439  619133  619141
  • UMLS: C3888102
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

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