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Frontotemporal dementia

Disease 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).

ORPHA:282

  • Synonym(s):
    • FTD
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: G31.0
  • OMIM: 172700  600274  600795  607485
  • UMLS: C0338451
  • MeSH: D057180
  • GARD: 8436
  • MedDRA: 10068968

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