Frontotemporal dementia


Authors: R. Rusina 1;  R. Matěj 2;  Z. Cséfalvay 3;  J. Keller 4;  V. Franková 5;  M. Vyhnálek 6,7
Authors‘ workplace: Neurologická klinika 3. LF UK a Fakultní Thomayerova nemocnice, Praha 1;  Ústav patologie a molekulární medicíny, 3. LF UK a Fakultní Thomayerova nemocnice, Praha 2;  Katedra logopedie, Komenského Univerzita, Bratislava, Slovensko 3;  Neurologická klinika, 3. LF UK a FN Královské Vinohrady, Praha 4;  Psychiatrická nemocnice v Dobřanech 5;  Neurologická klinika 2. LF UK a FN Motol, Praha 6;  Mezinárodní centrum klinického výzkumu, FN u sv. Anny, Brno 7
Published in: Cesk Slov Neurol N 2021; 84/117(1): 9-29
Category: Minimonography
doi: 10.48095/cccsnn20219

Overview

This minimonography presents a comprehensive view of frontotemporal dementia. The behavioral variant of frontotemporal dementia (bvFTD) is a clinical syndrome within the spectrum of frontotemporal lobar degenerations. Typical manifestations of bvFTD include early behavioral disturbance, personality changes, cognitive deficits with a dominant impairment of executive functions, and language impairment. MRI typically shows asymmetric atrophy with frontal lobe predominance. The underlying neuropathology is in up to one half of the cases of a tauopathy, while the most frequent tau negative inclusions are TDP-43 protein deposits. Many patients with amyotrophic lateral sclerosis may develop cognitive and behavioral manifestation similar to bvFTD; these disorders have recently been called amyotrophic lateral sclerosis – frontotemporal spectrum disorder. In conclusion, the most important differential diagnose are discussed: frontal variant of Alzheimer’s disease and psychiatric disorders mimicking bvFTD.

Keywords:

Frontotemporal dementia – frontotemporal lobar degeneration – Alzheimer’s disease – behavioral variant of frontotemporal dementia – Pick’s disease – tauopathy – TDP-43 proteinopathy


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