Differentiating between right-lateralised semantic dementia and behavioural-variant frontotemporal dementia: an examination of clinical characteristics and emotion processing
Open accessAbstract
BACKGROUND AND PURPOSE: Right-lateralised semantic dementia (right SD) and behavioural-variant frontotemporal dementia (bvFTD) appear clinically similar, despite different patterns of underlying brain changes. This study aimed to elucidate distinguishing clinical and cognitive features in right SD versus bvFTD, emphasising emotion processing and its associated neural correlates. METHODS: 12 patients with right SD and 19 patients with bvFTD were recruited. Clinical features were documented. All patients were assessed on standardised neuropsychological tests and a facial emotion processing battery. Performance was compared to 20 age-matched and education-matched controls. Grey matter intensity was related to emotion processing performance using whole-brain voxel-based morphometry analysis. RESULTS: Patients with right SD exhibited disproportionate language dysfunction, prosopagnosia and a suggestion of increased obsessive personality/behavioural changes versus patients with bvFTD. In contrast, patients with bvFTD demonstrated pronounced deficits in attention/working memory, increased apathy and greater executive dysfunction, compared to patients with right SD. Decreased empathy, disinhibition and diet changes were common to both dementia subtypes. Emotion processing deficits were present in both FTD syndromes but were associated with divergent patterns of brain atrophy. In right SD, emotion processing dysfunction was associated with predominantly right medial and lateral temporal integrity, compared to mainly left temporal, inferior frontal and orbitofrontal and right frontal gyrus integrity in bvFTD. CONCLUSIONS: This study demonstrates comparable deficits in facial emotion processing in right SD and bvFTD, in keeping with their similar clinical profiles. These deficits are attributable to divergent neural substrates in each patient group, namely, right lateralised regions in right SD, versus predominantly left lateralised regions in bvFTD.
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Differential diagnosis of sbvFTD remains challenging because of clinical overlap in socioemotional functioning with bvFTD and lexical semantic deficits with svPPA.
“Differential diagnosis of sbvFTD remains challenging because of the clinical overlap in socioemotional functioning with bvFTD and lexical semantic deficits with the left-lateralized semantic variant of primary progressive aphasia (svPPA).3,7,10”
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- Authors
- Kamminga, J., Kumfor, F., Burrell, J. R., Piguet, O., Hodges, J. R., & Irish, M.
- Affiliations
- UNSW Sydney (AU)
- Corresponding author
- Irish, Muireann
- Venue
- Journal of Neurology, Neurosurgery & Psychiatry
- Publisher
- BMJ
- Published
- 2014-12-15
- Volume
- 86
- Issue
- 10
- Pages
- 1082-1088
- ISSN
- 0022-3050 (ISSN-L)
- 1468-330X
- OpenAlex
- W2170304225
- Cited by
- 129
- References
- 5
- Citations by year
- 2026: 5 · 2025: 13 · 2024: 15 · 2023: 8 · 2022: 12 · 2021: 17 · 2020: 11 · 2019: 9 · 2018: 10 · 2017: 12 · 2016: 11 · 2015: 6
- FWCI
- 7.35
- Type
- journal article
- Language
- en
- Open access
- green
- handle.unsw.edu.au
- Licence
- cc-by-nc-nd
- Locations
- Journal of Neurology Neurosurgery & Psychiatry (publishedVersion)
- PubMed (publishedVersion)
- UWA Profiles and Research Repository (University of Western Australia) (submittedVersion)
- UNSWorks (UNSW Sydney) (submittedVersion, cc-by-nc-nd)
- UNSWorks (University of New South Wales, Sydney, Australia) (submittedVersion, cc-by-nc-nd)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Life Sciences › Neuroscience › Cognitive Neuroscience › Neurobiology of Language and Bilingualism
- Social Sciences › Psychology › Clinical Psychology › Historical and Modern Theater Studies
- Keywords
- behavioral variant frontotemporal dementia, emotional processing, facial emotion recognition, prosopagnosia, language disorders, apathy, executive dysfunction, gray matter atrophy, hemispheric lateralization, differential diagnosis, empathy deficits
- MeSH terms
- Aged, Atrophy, Brain, Cognition, Dementia, Diagnosis, Differential, Emotions, Face, Female, Humans, Functional Laterality, Magnetic Resonance Imaging, Male, Middle Aged, Neuropsychological Tests, Social Perception, Frontotemporal Dementia, Gray Matter
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