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Frontotemporal dementia

ORPHA:282

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.

Prevalence
1-9 / 100 000
Inheritance
Autosomal dominant
Age of onset
Adult