Fourteen years later: Reviewing the diagnostic criteria for behavioral-variant frontotemporal dementia

Olivier Piguet^{1

2}, Antonia J Clarke^{3

4}, David Foxe^{1

2}, Nga Yan Tse¹, Her Teng^{1

2}, James R Burrell^{1

3

5}, Sau Chi Cheung^{1

2}, James Carrick^{1

2}, Emma Devenney^{1

3}, Glenda M Halliday^{1

3}, John B Kwok^{1

3}, Carol Dobson-Stone^{1

3}, Nicholas J Cordato^{1

6

7

8}, Yun Tae Hwang^{1

9}, John R Hodges^{1

3}, Rebekah M Ahmed^{1

3

4}

Affiliations

¹ Brain and Mind Centre, The University of Sydney, Camperdown, New South Wales, Australia.
² School of Psychology, The University of Sydney, Camperdown, New South Wales, Australia.
³ Faculty of Medicine and Health, The University of Sydney, Camperdown, New South Wales, Australia.
⁴ Neuropsychology Unit, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia.
⁵ Concord Clinical School, Medical Education Centre, Concord General Hospital, The University of Sydney, Concord, New South Wales, Australia.
⁶ St. George Clinical School, University of New South Wales, Sydney, New South Wales, Australia.
⁷ The Department of Aged Care, St. George Hospital, Sydney, New South Wales, Australia.
⁸ Calvary Community Health, Calvary Health Care Kogarah, Sydney, New South Wales, Australia.
⁹ Department of Neurology, Gosford Hospital, Gosford, New South Wales, Australia.

PMID: 40820228
PMCID: PMC12358242
DOI: 10.1002/alz.70604

Fourteen years later: Reviewing the diagnostic criteria for behavioral-variant frontotemporal dementia

Olivier Piguet et al. Alzheimers Dement. 2025 Aug.

. 2025 Aug;21(8):e70604.

doi: 10.1002/alz.70604.

Authors

Affiliations

¹ Brain and Mind Centre, The University of Sydney, Camperdown, New South Wales, Australia.
² School of Psychology, The University of Sydney, Camperdown, New South Wales, Australia.
³ Faculty of Medicine and Health, The University of Sydney, Camperdown, New South Wales, Australia.
⁴ Neuropsychology Unit, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia.
⁵ Concord Clinical School, Medical Education Centre, Concord General Hospital, The University of Sydney, Concord, New South Wales, Australia.
⁶ St. George Clinical School, University of New South Wales, Sydney, New South Wales, Australia.
⁷ The Department of Aged Care, St. George Hospital, Sydney, New South Wales, Australia.
⁸ Calvary Community Health, Calvary Health Care Kogarah, Sydney, New South Wales, Australia.
⁹ Department of Neurology, Gosford Hospital, Gosford, New South Wales, Australia.

PMID: 40820228
PMCID: PMC12358242
DOI: 10.1002/alz.70604

Abstract

Introduction: Diagnosis of behavioral-variant frontotemporal dementia (bvFTD) requires the presence of at least three of six core features. The contribution of each criterion toward diagnosis, however, remains unclear. We aimed to determine the frequency and combinations of diagnostic criteria at presentation and their contribution to survival.

Methods: Consecutive patients with a stable diagnosis of bvFTD between 2010 and 2023 were included. Demographic and cognitive data were also collected.

Results: A total of 110 patients were identified. Frequency of diagnostic criteria ranged from 89% (apathy; empathy loss) to 14.5% (dysexecutive profile), and 78 patients met at least four criteria. Mean survival time from symptom onset was 10.4 years and was not affected by the number and combination of core diagnostic criteria (P values ≥ 0.274).

Discussion: Our findings show that the requirements for the cognitive criterion are disproportionately restrictive, which may delay diagnosis and access to relevant treatments and therapies.

Highlights: The comparative usefulness of core criteria for frontotemporal dementia diagnosis is unclear. We identified the most common profiles of core features at baseline. Most common feature profiles included apathy and/or empathy loss. Feature profiles were not predictive of survival. Cognitive criterion appears overly restrictive in its current form.

Keywords: behavior; behavioral‐variant frontotemporal dementia; clinical presentation; cognition; survival.

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Conflict of interest statement

The authors declare no conflicts of interest. Author disclosures are available in the supporting information.

Figures

**FIGURE 1**
Frequencies of core diagnostic criteria for behavioral‐variant frontotemporal dementia. Each bar represents the frequency (%) of the core diagnostic criteria at presentation

**FIGURE 2**
Frequencies of combinations of core diagnostic criteria for behavioral‐variant frontotemporal dementiain sporadic and genetic cases

**FIGURE 3**
Core cognitive criterion (F) with and without concurrent exclusion features (episodic memory disturbance; visuospatial disturbance) in patients diagnosed with behavioral‐variant frontotemporal dementia

**FIGURE 4**
Mean disease survival from disease onset in male and female patients diagnosed with behavioral‐variant frontotemporal dementia

See this image and copyright information in PMC

References

1. Bang J, Spina S, Miller BL. Frontotemporal dementia. Lancet. 2015;386:1672‐1682. doi: 10.1016/s0140-6736(15)00461-4 - DOI - PMC - PubMed
1. van den Stock J, Bertoux M, Diehl‐Schmid J, et al. Current potential for clinical optimization of social cognition assessment for frontotemporal dementia and primary psychiatric disorders. Neuropsychol Rev. 2023;33:544‐550. doi: 10.1007/s11065-022-09554-3 - DOI - PubMed
1. Ahmed RM, Hodges JR, Piguet O. Frontotemporal dementias: emerging milestones of the 21st century. Adv Exp Med Biol. 2021;1281:1‐15. doi: 10.1007/978-3-030-51140-1_1 - DOI - PubMed
1. Piguet O, Kumfor F, Hodges JR. Diagnosing, monitoring and managing behavioural variant frontotemporal dementia. Med J Austral. 2017;207:303‐308. doi: 10.5694/mja16.01458 - DOI - PubMed
1. Boeve BF, Boxer AL, Kumfor F, Pijnenburg Y, Rohrer JD. Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations. Lancet Neurol. 2022;21:258‐272. doi: 10.1016/s1474-4422(21)00341-0 - DOI - PubMed

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Fourteen years later: Reviewing the diagnostic criteria for behavioral-variant frontotemporal dementia

Affiliations

Fourteen years later: Reviewing the diagnostic criteria for behavioral-variant frontotemporal dementia

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Miscellaneous