The pathological basis of semantic dementia
Abstract
Semantic dementia is a syndrome of progressive deterioration in semantic memory (knowledge of objects, people, concepts and words). It falls within the clinical spectrum of frontotemporal dementia but its pathology is yet to be studied systematically. This study included 18 consecutive post mortem cases meeting clinical criteria for semantic dementia. Clinic records and diagnostic histopathology were available for all cases; structural neuroimaging, neuropsychology and semi-quantitative histopathology/immunohistochemistry data were analysed where possible. The pathological diagnosis in a clear majority of cases was frontotemporal degeneration with ubiquitin inclusions (n = 13). Eleven of these cases had characteristic motor neuron disease-type inclusions in the dentate gyrus and cerebral cortex. Ubiquitin inclusions were found only in the inferior olivary nucleus in the other two, one of which was the only case to show degeneration of motor tracts and also to have shown evidence of motor neuron disease during life. None of the patients had motor symptoms or signs at presentation. A family history of motor neuron disease was documented in one case. Pick body-positive Pick's disease appeared three times. Two cases had Alzheimer's disease and significant coincidental Alzheimer-type pathology was also found in one of the ubiquitin inclusion cases. One of the Alzheimer's disease patients had changes in white matter signal on scanning, whereas all other scans showed cerebral atrophy only. Semi-quantitative assessment of regional neuronal loss found that anterior and inferior temporal regions bore the brunt of disease across all histopathological subtypes, usually on the left side, implicating this region in semantic processing.
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A severe neuronal loss in the CA1 subfield together with a preservation of neurons in the dentate gyrus, consistent with our imaging findings, was reported independently of the histopathological subtypes.
“Despite this variability, a severe neuronal loss in the CA1 subfield together with a preservation of neurons in the dentate gyrus, consistent with our imaging findings, was reported independently of the histopathological subtypes (Davies et al., 2005).”
From Hippocampal subfield volumetry in mild cognitive impairment, Alzheimer's disease and semantic dementia (2013) · cites this paper as Davies et al., 2005
The pathological substrate of hippocampal atrophy in SD is not clear, notably because patients with SD can present with heterogeneous pathological features.
“Yet, the pathological substrate of hippocampal atrophy in SD is not clear, notably because patients with SD can present with heterogeneous pathological features (Davies et al., 2005).”
From Hippocampal subfield volumetry in mild cognitive impairment, Alzheimer's disease and semantic dementia (2013) · cites this paper as Davies et al., 2005
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- Authors
- Davies, R. R., Hodges, J. R., Kril, J. J., Patterson, K., Halliday, G. M., & Xuereb, J. H.
- Affiliations
- University of Cambridge (GB)
- MRC Cognition and Brain Sciences Unit (GB)
- The University of Sydney (AU)
- UNSW Sydney (AU)
- Corresponding author
- Davies, R. Rhys
- ORCID
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 2005-07-06
- Volume
- 128
- Issue
- 9
- Pages
- 1984-1995
- ISSN
- 0006-8950 (ISSN-L)
- 1460-2156
- OpenAlex
- W2137466608
- Cited by
- 325
- References
- 62
- Citations by year
- 2026: 3 · 2025: 7 · 2024: 3 · 2023: 6 · 2022: 9 · 2021: 13 · 2020: 8 · 2019: 11 · 2018: 18 · 2017: 11 · 2016: 12 · 2015: 9 · 2014: 16 · 2013: 18 · 2012: 19
- FWCI
- 16.24
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Brain (publishedVersion)
- PubMed (publishedVersion)
- Dkgest of the World Latest Medical Information
- Funders
- Wellcome Trust
- Medical Research Council
- National Health and Medical Research Council
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- semantic dementia, frontotemporal dementia, Pick's disease, Alzheimer's disease, motor neuron disease, dentate gyrus, inferior olive, neuronal loss, temporal lobe, semantic memory, postmortem neuropathology
- MeSH terms
- Aged, Aged, 80 and over, Alzheimer Disease, Brain, Dementia, Female, Humans, Male, Middle Aged, Neuropsychological Tests, Semantics, Temporal Lobe, Pick Disease of the Brain, Ubiquitin
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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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