Frontotemporal dementia

doi:10.14735/amcsnn20209

Frontotemporal dementia

2021-01-01

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DOI：10.14735/amcsnn20209

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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

Frontotemporal dementia

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