P4‐166: Determinants of survival in autopsy‐confirmed patients with behavioral variant frontotemporal dementia (bvFTD): Second Report of the international bvFTD criteria consortium (FTDC)
Bibliographic record
Abstract
Few studies have explored determinants of survival in behavioral-variant frontotemporal dementia (bvFTD). This information is important, as it can inform clinical trials and provide prognostic guidelines for patients and their families. The present study explored survival in a multi-site sample of bvFTD patients with confirmed FTLD pathology. 148 cases from the International bvFTD Criteria Consortium (FTDC) database were included. Cases met Possible bvFTD criteria at presentation and had FTLD pathology at autopsy. Kaplan Meier and Cox hazards analyses were used to determine demographic, behavioral and neurological factors affecting survival. The total sample was male predominant (61 females, 87 males) with a mean age of 57 (10.2) at initial evaluation. Median survival from disease onset was 7 years (95% CI: 6.4-7.7), and median survival was 3 years from initial evaluation (95% CI: 2.4-3.6). Kaplan Meier analyses revealed that presence of motor neuron disease (MND), extrapyramidal symptoms, motor speech deficits and disinhibition were significantly associated with reduced survival from initial evaluation, while tau-positive pathology was associated with longer overall survival. Education, family history of an FTD spectrum disorder, MMSE scores, age at onset and age at initial presentation did not significantly affect survival from initial evaluation. Excluding cases with MND (n = 26; median survival 2 years from initial evaluation), median survival increased to 8 years from disease onset (95% CI: 7.3-8.6), and 4 years from initial evaluation (95% CI: 3.4-4.6). Extrapyramidal symptoms and disinhibition remained significant predictors of decreased survival, with reduced survival in males compared to females. However, motor speech deficits and tau pathology were no longer associated with survival when excluding cases with MND. BvFTD is associated with a poor prognosis. Our findings suggest that presence of MND, extrapyramidal symptoms and disinhibition negatively impact survival in bvFTD.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".