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https://doi.org/10.22364/lmahc.fd.24.01 | 6-19 | PDF

Frontotemporal Degeneration

Katrin Gross-Paju
Tallinn University of Technology

Frontotemporal degeneration (FTD) is a clinically, genetically and neuropathologically heterogeneous syndrome. The clinical syndromes of FTD are typically characterized by progressive aphasia, decline in executive functions and behavioural problems. Recently, several other phenotypes have been described, such as progressive supranuclear palsy (PSP) and the corticobasal syndrome (CBS), frequently in combinations. Furthermore, the inclusion body myopathy may be a rare feature of FTD. FTD is the most frequent early-onset (<65 years) dementia, according to different studies comprising 3–26% of all dementias. Although FTD is commonly addressed as young-onset dementia, the highest number of patients is diagnosed in the age range from 70 to 84, and the highest incidence at the age of 71, confirming the need to consider FTD in characteristic phenotypes in different age groups. Positive family history is present in 25–50% of FTD, mostly in autosomal dominant pattern, and therefore genetic studies are indicated, if FTD phenotype is present.

Keywords: frontotemporal degeneration, dementia, aphasia, behavioural changes

From: Balode, Ineta; Lele-Rozentāle, Dzintra; Priede, Zanda (compilers and scientific editors). Linguistic and Medical Aspects in Health Care. Focus on Dementia. Riga: University of Latvia Press, 2024.