Links Between Frontotemporal Lobar Degeneration, Corticobasal Degeneration, Progressive Supranuclear Palsy, and Amyotrophic Lateral Sclerosis
Abstract
Frontotemporal lobar degeneration, corticobasal degeneration (CBD), progressive supranuclear palsy, and amyotrophic lateral sclerosis have been considered distinct clinicopathologic entities with few issues in common other than neurodegeneration being central to all. The aim of this paper is to highlight the clinical, topographic, pathologic, proteomic, and genetic similarities among these disorders and the syndromes through which each disorder is exhibited. The critical roles of tau and TAR DNA-binding protein 43 (TDP-43) dysfunction in the disorders and syndromes are emphasized. Although confusion certainly remains, and the ability to predict the underlying proteinopathy in the various neurodegenerative syndromes is far from perfect, there is optimism that insights gained over the next few years will enhance our ability to accurately identify the amyloidopathies, tauopathies, and TDP-43opathies early in the disease course, potentially improving the ability to impact these diseases once targeted therapies have been developed.
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The clinical syndrome of PSP highly correlates with PSP tau pathology.
“The clinical syndrome of PSP highly correlates with PSP tau pathology.”
From New developments of biofluid‐based biomarkers for routine diagnosis and disease trajectories in frontotemporal dementia (2022) · cites this paper as [26]
The presence of svPPA and FTD-ALS shows a strong correlation with TDP-43 pathology.
“The presence of svPPA and FTD‐ALS show a strong correlation with TDP‐43 pathology.”
From New developments of biofluid‐based biomarkers for routine diagnosis and disease trajectories in frontotemporal dementia (2022) · cites this paper as [26]
Almost half of bvFTD have an underlying FTLD-tau pathology, including PiD and to a lesser extent CBD and PSP pathologies.
“Almost half of bvFTD have an underlying FTLD‐tau pathology, including PiD and to lesser extent CBD and PSP pathologies.”
From New developments of biofluid‐based biomarkers for routine diagnosis and disease trajectories in frontotemporal dementia (2022) · cites this paper as [26]
Parkinsonism in association with bvFTD or PPA is a predictor of tau pathology.
“In addition, parkinsonism in association with bvFTD or PPA is a predictor of tau pathology.”
From New developments of biofluid‐based biomarkers for routine diagnosis and disease trajectories in frontotemporal dementia (2022) · cites this paper as [26]
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- Authors
- Boeve, B. F.
- Affiliations
- Alzheimer's Association (US)
- Corresponding author
- Boeve, Bradley F.
- ORCID
- Boeve, Bradley F.
- Venue
- Alzheimer Disease & Associated Disorders
- Published
- 2007-10
- Volume
- 21
- Issue
- 4
- Pages
- S31-S38
- ISSN
- 0893-0341 (ISSN-L)
- OpenAlex
- W2007785503
- Cited by
- 73
- References
- 58
- Citations by year
- 2026: 1 · 2025: 7 · 2024: 1 · 2023: 2 · 2022: 2 · 2021: 5 · 2020: 2 · 2018: 7 · 2017: 1 · 2016: 2 · 2015: 4 · 2014: 4 · 2013: 8 · 2012: 1
- FWCI
- 0.65
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Alzheimer Disease & Associated Disorders (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Mayo Foundation for Medical Education and Research
- Topics
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Keywords
- frontotemporal lobar degeneration, corticobasal degeneration, progressive supranuclear palsy, amyotrophic lateral sclerosis, tauopathy, tau protein, TDP-43, neurodegeneration, proteinopathies, early diagnosis, genetic similarity
- MeSH terms
- Amyotrophic Lateral Sclerosis, Dementia, Humans, Supranuclear Palsy, Progressive, Neurodegenerative Diseases
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