The evolution and pathology of frontotemporal dementia
Abstract
This is a clinicopathologic study of a prospective, clinic-based cohort of patients with frontotemporal dementia (FTD)/Pick complex, who were followed to autopsy. A total of 60 patients with the clinical syndromes of the behavioural variant of FTD (FTD-bv) (n = 32), primary progressive aphasia (PPA) (n = 22), corticobasal degeneration syndrome (CBDS) (n = 4) and progressive supranuclear palsy (PSP) (n = 2) at onset, referred to a cognitive neurology clinic who had subsequent post-mortem examination were included. The most common histological variety was motor neurone disease type inclusion (MNDI) (n = 18), followed by corticobasal degeneration (CBD) (n = 12), then Pick's disease (n = 6), dementia lacking distinctive histology (DLDH) (n = 6) and PSP (n = 3). Others fulfilled the histological criteria for Alzheimer's disease combined with glial pathology (n = 6), Alzheimer's disease only (n = 4), Lewy body variant (n = 2), prion disease (n = 1), vascular dementia (n = 1) and undetermined (n = 1). The most common first syndrome among the MNDI and DLDH (tau negative) pathologies was FTD-bv, but subsequently progressive aphasia (PA), occasionally CBDS and semantic dementia also developed. Tau positive histologies of CBD, PSP and Pick bodies were most frequently associated with PPA onset or CBDS/PSP, but behavioural symptoms were also common. Age of onset was earlier in tau negative cases, but the duration of illness and gender distribution were about the same in all histological variants. Although the tau negative and positive histologies are predicted to some extent by the clinical onset, the extent of the overlap and the convergence of the syndromes in the course of the disease argue in favour of maintaining the clinical and pathological varieties under a single umbrella.
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The proportion of fvAD is likely higher because AD pathology can be found in up to 25% of patients with a clinical diagnosis of bvFTD.
“although this figure is likely higher because AD pathology can be found in up to 25% of patients with a clinical diagnosis of bvFTD [9, 10].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [9]
The differential diagnosis of frontotemporal dementia (FTD) remains challenging due to clinical and pathological heterogeneity and symptomatic overlap with other neurodegenerative and psychiatric disorders.
“Due to its clinical and pathological heterogeneity and symptomatic overlap with other neurodegenerative diseases, such as Alzheimer’s disease (AD), and other non-neurodegenerative psychiatric disorders, the differential diagnosis of FTD remains challenging [2, 7–9].”
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“Furthermore, FTLD consists of multiple different conditions with distinct underlying pathologies(2) that have overlapping clinical features.(3) Therefore, the clinical diagnosis of frontotemporal dementia syndrome (FTD), or frontotemporal dementia spectrum disorder, is sometimes used.”
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- Authors
- Kertesz, A., McMonagle, P., Blair, M., Davidson, W., & Munoz, D. G.
- Affiliations
- St. Joseph's Hospital (CA)
- Corresponding author
- Kertesz, Andrew
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 2005-07-20
- Volume
- 128
- Issue
- 9
- Pages
- 1996-2005
- ISSN
- 0006-8950 (ISSN-L)
- 1460-2156
- OpenAlex
- W2102286051
- Cited by
- 609
- References
- 40
- Citations by year
- 2026: 1 · 2025: 13 · 2024: 15 · 2023: 13 · 2022: 9 · 2021: 24 · 2020: 23 · 2019: 28 · 2018: 11 · 2017: 30 · 2016: 33 · 2015: 28 · 2014: 36 · 2013: 47 · 2012: 28
- FWCI
- 26.67
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Brain (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- frontotemporal dementia, behavioral variant frontotemporal dementia, primary progressive aphasia, corticobasal degeneration, progressive supranuclear palsy, Pick's disease, tau pathology, disease progression, clinicopathological correlation, histological subtypes
- MeSH terms
- Adult, Aged, Alzheimer Disease, Dementia, Female, Humans, Male, Middle Aged, Prospective Studies, Supranuclear Palsy, Progressive, Syndrome, Survival Analysis, tau Proteins, Age of Onset, Sex Distribution, Disease Progression, Aphasia, Primary Progressive, Pick Disease of the Brain
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