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Dementia is a collective term for cognitive disorders primarily affecting memory, thinking, and reasoning. It is not a specific disease but a syndrome, with Alzheimer's disease being the most common cause, accounting for approximately 60-80% of cases. Other types include vascular dementia, Lewy body dementia, and frontotemporal dementia. Dementia affects millions worldwide, particularly older adults, though it is not a normal part of aging.
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Prosopagnosia, also known as face blindness, is the inability to recognize faces. In severe cases, individuals with prosopagnosia may not recognize close family members, including parents and spouses, by their faces. For instance, someone with prosopagnosia might walk past their child in a crowd, only realizing their mistake upon noticing their child's distinctive backpack or favorite jacket. Prosopagnosia specifically impairs facial recognition, while the recognition of other objects or...
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Frontotemporal Dementia.

David Glenn Clark

    Continuum (Minneapolis, Minn.)
    |December 2, 2024
    PubMed
    Summary

    Frontotemporal dementia (FTD) is a complex brain disorder with three core syndromes. Understanding FTD pathology, including protein derangements and genetic variations, aids diagnosis and treatment development.

    Area of Science:

    • Neuroscience
    • Genetics
    • Neurology

    Background:

    • Frontotemporal dementia (FTD) encompasses diverse clinical syndromes, with three core types: behavioral variant FTD, nonfluent primary progressive aphasia, and semantic primary progressive aphasia.
    • Clinical manifestations correlate with specific brain network involvement.
    • The underlying pathology is primarily attributed to derangements in three proteins: transactive response DNA-binding protein 43 (TDP-43), MAPT, and FUS.

    Purpose of the Study:

    • To present a simplified framework for understanding frontotemporal dementia (FTD) syndromes.
    • To detail the pathology and genetic variations associated with FTD.
    • To provide clinicians with a foundation for diagnosing and managing FTD.

    Main Methods:

    • Review and synthesis of current knowledge on FTD clinical presentations, pathology, and genetics.

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  • Classification of FTD into three core syndromes based on clinical features and underlying pathophysiology.
  • Discussion of genetic factors, including C9orf72, MAPT, and GRN variations.

    • Main Results:

    • FTD syndromes can be broadly categorized into behavioral variant FTD and two forms of primary progressive aphasia.
    • TDP-43, MAPT, and FUS proteins are implicated in the majority of FTD cases.
    • Genetic variations in C9orf72, MAPT, and GRN are significant contributors to FTD heritability, found in over 10% of cases.

    Conclusions:

    • A simplified framework aids in understanding the heterogeneity of FTD.
    • Currently, no disease-modifying treatments exist, but clinical trials targeting genetic variations are ongoing.
    • Biomarker advancements are crucial for accelerating the development of novel pharmacologic treatments for FTD.