Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia
Abstract
We conducted a French multicentric cross-sectional study to describe in detail the demographic, neurological and behavioural characteristics of the frontal variant of frontotemporal dementia (fvFTD) and to characterize the pattern of brain perfusion SPECT in comparison to a healthy control group. A total of 68 fvFTD patients had technetium-99m-ECD brain perfusion SPECT at inclusion, 61 of which also underwent an in-depth evaluation including 70 items assessing behaviour, language and affect/emotion at onset and at inclusion. The mean age-at-onset was 60.4 +/- 7.8 years (35-75). Twenty-six per cent of the patients were older than 65 at onset. A positive familial history consistent with an autosomal dominant inheritance was found in 18% of the patients. At onset, the behavioural profile was predominantly inert in 25% of the patients, disinhibited in 18% and mixed in others. The behavioural features progressed to predominantly mixed or inert forms. Although, inertia was associated with predominant medial frontal and cingulate hypoperfusion, and patients with disinhibition exhibited predominant ventromedial prefrontal and temporal hypoperfusion, there were no major clinical differences between disinhibited and inert patients. Forty-five per cent of the deceased patients survived 8 years (long survival). This shows that the final outcome of fvFTD is highly variable. No clinical factors predictive of short or long survival were identified. Unexpected, however, was the finding that…
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Specific brainstem hypoperfusion associated with fast clinical progression in bvFTD.
“specific brainstem hypoperfusion that associated with fast clinical progression in bvFTD (Le Ber et al., 2006)”
From The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials (2024) · cites this paper as Le Ber et al., 2006
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Association Between Tau H2 Haplotype and Age at Onset in Frontotemporal Dementia
Borroni, B., Yancopoulou, D., Tsutsui, M., Padovani, A., Sawcer, S. J., Hodges, J. R., & Spillantini, M. G. (2005) · Archives of Neurology · doi:10.1001/archneur.62.9.1419 · Cited by 38 · see citing works
Metabolic correlates of behavioral and affective disturbances in frontal lobe pathologies
Sarazin, M., Michon, A. E. S., Pillon, B., Samson, Y., Canuto, A., Gold, G., Bouras, C., Dubois, B., & Giannakopoulos, P. (2003) · Journal of Neurology · doi:10.1007/s00415-003-1087-z · Cited by 32 · see citing works
Self-citationFrontotemporal dementia: a clinicopathological review of four postmortem studies
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Frontal lobe degeneration: clinical, neuropsychological and SPECT characteristics
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Sporadic and Familial Dementia With Ubiquitin-Positive Tau-Negative Inclusions
Godbolt, A. K., Josephs, K. A., Révész, T., Warrington, E. K., Lantos, P. L., King, A., Fox, N. C., Sarraj, S. A., Holton, J. L., Lisa Cipolotti, A., et al. (2005) · Archives of Neurology · doi:10.1001/archneur.62.7.1097 · Cited by 26 · see citing works
Similar early clinical presentations in familial and non-familial frontotemporal dementia
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L'épreuve de rappel indicé à 48 items (RI-48)
Adam, S., der Linden, M. V., Poitrenaud, J., et al. (2004) · Open Repository and Bibliography (University of Liège) · Cited by 8 · see citing works
Flagged first, then by citations
Cited by
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Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia
Rascovsky, K., Hodges, J. R., Knopman, D., Mendez, M. F., Kramer, J. H., Neuhaus, J., van Swieten, J. C., Seelaar, H., Dopper, E. G. P., Onyike, C. U., et al. (2011) · Brain · doi:10.1093/brain/awr179 · Cited by 5,221 · see citing works
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Clinical, genetic and pathological heterogeneity of frontotemporal dementia: A review
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A critical role for the hippocampus in the valuation of imagined outcomes
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Self-citationApathy: A neurocircuitry model based on frontotemporal dementia
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Predicting functional decline in behavioural variant frontotemporal dementia
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What Do We Mean by Behavioral Disinhibition in Frontotemporal Dementia?
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Psychosis in behavioral variant frontotemporal dementia
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Fieldhouse, J. L. P., van Paassen, D. N., van Engelen, M.-P. E., De Boer, S. C. M., Hartog, W. L., Braak, S., Schoonmade, L. J., Schouws, S. N. T. M., Krudop, W. A., Oudega, M. L., et al. (2024) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2024.1382593 · Cited by 8 · see citing works
Trajectories of behavioural disturbances across dementia types
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- Authors
- Ber, I. L., Guedj, E., Gabelle, A., Verpillat, P., Volteau, M., Thomas-Anterion, C., Decousus, M., Hannequin, D., Vera, P., Lacomblez, L., Camuzat, A., Didic, M., Puel, M., Lotterie, J.-A., Golfier, V., Bernard, A.-M., Vercelletto, M., Magne, C., Sellal, F., Namer, I., Michel, B.-F., Pasquier, J., Salachas, F., Bochet, J., Brice, A., Habert, M.-O., & Dubois, B.
- Affiliations
- Centre Hospitalier de Saint-Quentin (FR)
- Hôpital de la Timone (FR)
- Assistance Publique – Hôpitaux de Paris (FR)
- Inserm (FR)
- Hôpital Rangueil (FR)
- Centre Hospitalier de Saint-Brieuc (FR)
- Centre Eugène Marquis (FR)
- Centre Hospitalier Universitaire de Nantes (FR)
- Hôpital d'Hautepierre (FR)
- ORCID
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 2006-09-29
- Volume
- 129
- Issue
- 11
- Pages
- 3051-3065
- ISSN
- 0006-8950 (ISSN-L)
- 1460-2156
- OpenAlex
- W2077311804
- Cited by
- 179
- References
- 108
- Citations by year
- 2026: 2 · 2025: 2 · 2024: 10 · 2023: 5 · 2022: 12 · 2021: 19 · 2020: 14 · 2019: 6 · 2018: 5 · 2017: 11 · 2016: 10 · 2015: 8 · 2014: 7 · 2013: 14 · 2012: 7
- FWCI
- 5.10
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Brain (publishedVersion)
- PubMed (publishedVersion)
- HAL (submittedVersion)
- HighWire Press Open Archive (submittedVersion)
- Funders
- Institut National de la Santé et de la Recherche Médicale
- GIS-Institut des Maladies Rares
- 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
- brain perfusion SPECT, technetium-99m ECD, cerebral hypoperfusion, disinhibition, survival outcomes, prognostic factors, family history (medicine), autosomal dominant inheritance, ventromedial prefrontal cortex, cingulate cortex
- MeSH terms
- Adult, Aged, Brain, Brain Mapping, Brain Stem, Cerebrovascular Circulation, Cross-Sectional Studies, Dementia, Female, Humans, Male, Middle Aged, Neuropsychological Tests, Prognosis, Social Behavior Disorders, Tomography, Emission-Computed, Single-Photon, Survival Analysis, Age of Onset, Disease Progression
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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