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Frontotemporal dementia

Orpha number ORPHA282
Synonym(s) FTD
Prevalence 1-9 / 100 000
Inheritance Autosomal dominant
Age of onset Adult
ICD-10
  • G31.0
ICD-O -
OMIM
UMLS
  • C0338451
MeSH
  • D057180
MedDRA
  • 10068968

Summary

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).


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Detailed information

Article for general public
  • FR (2007,pdf)
Clinical genetics review
  • EN (2012)
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