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Dementia is an acquired, progressive syndrome characterized by a decline in multiple cognitive domains severe enough to impair daily functioning and reduce independence. Although memory loss is a central feature, the diagnosis requires additional deficits involving language, executive function, visuospatial skills, judgment, calculation, or abstract reasoning. These cognitive impairments reflect underlying neurodegenerative or vascular processes that gradually disrupt neuronal networks...
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Abbiategrasso Brain Bank Protocol for Collecting, Processing and Characterizing Aging Brains
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Published on: June 3, 2020

Frontotemporal degeneration.

Görsev G Yener, Howard J Rosen, John Papatriantafyllou

    Continuum (Minneapolis, Minn.)
    |July 20, 2012
    PubMed
    Summary
    This summary is machine-generated.

    Frontotemporal degeneration (FTD) is a common cause of dementia, with subtypes like bvFTD, SV, and PNFA. Research is advancing treatments for FTD, with clinical trials anticipated soon.

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    Area of Science:

    • Neurology
    • Neuroscience
    • Pathology

    Background:

    • Frontotemporal degeneration (FTD) is a leading cause of dementia in younger individuals.
    • FTD encompasses subtypes including behavioral variant FTD (bvFTD), semantic variant (SV), and progressive nonfluent aphasia (PNFA).
    • Related disorders like corticobasal degeneration and progressive supranuclear palsy share clinical and pathological links with FTD.

    Purpose of the Study:

    • To review the clinical spectrum, underlying pathology, and diagnostic imaging findings of Frontotemporal Degeneration (FTD).
    • To highlight the genetic basis and emerging therapeutic investigations for FTD.

    Main Methods:

    • Review of clinical presentations, pathological hallmarks (tau and TDP-43 proteinopathies), and genetic factors (chromosome 17 mutations).
    • Analysis of neuroimaging findings, including atrophy and hypometabolism patterns specific to FTD subtypes.
    • Summary of current research into FTD treatments.

    Main Results:

    • FTD presents with diverse symptoms including behavioral changes, psychiatric issues, aphasia, and parkinsonism.
    • Pathological examination reveals tau and TAR DNA-binding protein 43 (TDP-43) proteinopathies in most FTD cases.
    • Genetic mutations in tau and progranulin genes are identified in 5-10% of familial FTD.
    • Distinct imaging patterns are observed: bifrontal in bvFTD, temporal in SV, and perisylvian in PNFA.

    Conclusions:

    • FTD is a complex neurodegenerative disorder with varied clinical and pathological profiles.
    • Neuroimaging plays a crucial role in differentiating FTD subtypes.
    • Active research is paving the way for potential clinical trials and novel treatments for FTD.