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Frontotemporal dementia (FTD) has long been viewed as a strongly heritable condition. However, many familial cases do not involve known mutations. To identify the extent to which known genes account for the heritability of FTD spectrum disorders, researchers rated the strength of the family history in 225 patients with an FTD spectrum clinical diagnosis and performed extensive genotyping, screening for mutations in genes commonly and uncommonly associated with FTD, including the genes for progranulin (PGRN), microtubule-associated protein tau (MAPT), TDP-43 (TARDP) and fused in sarcoma (FUS).
Only 10% of participants had clear autosomal dominant inheritance, but 42% had some family history. The behavioral variant of FTD was the most heritabl…