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Frontotemporal lobar degeneration: a clinical approach
Elissaios Karageorgiou1, Bruce L Miller1
1Memory and Aging Center, Department of Neurology, University of California San Francisco, San Francisco, California.
Seminars in Neurology
|June 26, 2014
Summary
Frontotemporal lobar degeneration (FTLD) involves frontal and temporal lobe atrophy, presenting as three syndromes: behavioral variant FTD (bvFTD), nonfluent, and semantic primary progressive aphasia (PPA). Management and emerging treatments are discussed.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Frontotemporal lobar degeneration (FTLD) is a significant cause of dementia, characterized by frontal and temporal lobe atrophy and abnormal protein aggregates.
- It represents approximately 10% of pathologically confirmed dementia cases.
- FTLD encompasses three distinct clinical syndromes: behavioral variant frontotemporal dementia (bvFTD), nonfluent-agrammatic primary progressive aphasia (nfvPPA), and semantic variant PPA (svPPA).
Purpose of the Study:
- To provide a comprehensive clinical approach to frontotemporal lobar degeneration (FTLD).
- To detail the differential impairments, associated atrophy patterns, and underlying proteinopathies for each FTD syndrome.
- To discuss current management strategies and promising future therapeutic avenues for FTD.
Main Methods:
- Review of clinical presentations, diagnostic features, and neuropathological substrates of FTD syndromes.
- Correlation of clinical syndromes with specific patterns of brain atrophy and underlying proteinopathies (tau, TDP-43, FUS).
- Analysis of genetic mutations (MAPT, GRN, C9Orf72) associated with FTD and Parkinsonism.
Main Results:
- Each FTD syndrome exhibits distinct early impairments in behavioral, executive, or language functions, with relative sparing of memory and visuospatial abilities.
- Specific atrophy patterns (frontal for bvFTD/nfvPPA, temporal for svPPA) provide unique imaging signatures.
- Parkinsonism is associated with tau pathology and MAPT/GRN mutations; bvFTD co-occurs with motor neuron disease in some cases, often linked to C9Orf72 mutations.
Conclusions:
- FTLD presents with distinct clinical syndromes (bvFTD, nfvPPA, svPPA) linked to specific neuroanatomical and pathological features.
- Management involves symptomatic treatment, with caution regarding antipsychotics.
- Emerging treatments targeting underlying pathologies, such as anti-tau antibodies and progranulin enhancers, offer future therapeutic promise.
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