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.