SEMANTIC DEMENTIA: PROGRESSIVE FLUENT APHASIA WITH TEMPORAL LOBE ATROPHY
Abstract
We report five patients with a stereotyped clinical syndrome characterized by fluent dysphasia with severe anomia, reduced vocabulary and prominent impairment of single-word comprehension, progressing to a stage of virtually complete dissolution of the semantic components of language. A marked reduction in the ability to generate exemplars from restricted semantic categories (e.g. animals, vehicles, etc.) was a consistent and early feature. Tests of semantic memory demonstrated a radically impoverished knowledge about a range of living and man-made items. In contrast, phonology and grammar of spoken language were largely preserved, as was comprehension of complex syntactic commands. Reading showed a pattern of surface dyslexia. Autobiographical and day-to-day (episodic) memory were relatively retained. Non-verbal memory, perceptual and visuospatial abilities were also strikingly preserved. In some cases, behavioural and personality changes may supervene; one patient developed features of the Kluver-Bucy Syndrome. Radiological investigations have shown marked focal temporal atrophy in all five patients, and functional imaging by single positron emission tomography and positron emission tomography (one case) have implicated the dominant temporal lobe in all five. In the older literature, such cases would have been subsumed under the rubric of Pick's disease. Others have been included in series with progressive aphasia. We propose the term semantic dementia, first coined by Snowden et al. (1989), to designate this clinical syndrome.
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The management of semantic memory involves multiple brain regions, such as the left prefrontal cortex (LPC) being involved in the retrieval of semantic information, and the temporal lobes storing specific information related to objects.
“The management of semantic memory involves multiple brain regions, such as the left prefrontal cortex (LPC) being involved in the retrieval of semantic information, and the temporal lobes storing specific information related to objects [80, 81, 82].”
From Human-inspired Perspectives: A Survey on AI Long-term Memory (2024) · cites this paper as [80]
Previous attempts to differentiate FTD from AD on the basis of reduced concentration and executive dysfunction or reduced memory have often shown uncertain or even contradictory results.
“Previous attempts to differentiate frontotemporal dementia (FTD) from Alzheimer’s disease (AD) on the basis of reduced concentration and executive dysfunction or reduced memory have often shown uncertain or even contradictory results [5]”
From Alzheimer's Disease and Frontotemporal Dementia: A Robust Classification Method of EEG Signals and a Comparison of Validation Methods (2021) · cites this paper as [5]
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- Authors
- HODGES, J. R., PATTERSON, K., OXBURY, S., & FUNNELL, E.
- Affiliations
- University of Cambridge (GB)
- University of Oxford (GB)
- University of London (GB)
- Corresponding author
- HODGES, JOHN R.
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 1992
- Volume
- 115
- Issue
- 6
- Pages
- 1783-1806
- ISSN
- 0006-8950 (ISSN-L)
- 1460-2156
- OpenAlex
- W2033823029
- Cited by
- 1,710
- References
- 0
- Citations by year
- 2026: 23 · 2025: 23 · 2024: 30 · 2023: 31 · 2022: 36 · 2021: 43 · 2020: 47 · 2019: 48 · 2018: 50 · 2017: 47 · 2016: 60 · 2015: 64 · 2014: 62 · 2013: 56 · 2012: 55
- FWCI
- 19.03
- 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
- Life Sciences › Neuroscience › Cognitive Neuroscience › Neurobiology of Language and Bilingualism
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- semantic dementia, semantic memory impairment, anomia, Pick's disease, progressive aphasia, temporal lobe atrophy, Klüver-Bucy syndrome, surface dyslexia, positron emission tomography
- MeSH terms
- Adult, Aged, Aphasia, Atrophy, Dementia, Female, Humans, Language Disorders, Magnetic Resonance Imaging, Male, Memory, Middle Aged, Semantics, Temporal Lobe, Visual Perception
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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