Survival in Primary Progressive Aphasia: Comparison with bvFTD and FTD-ALS (P1.217)
Bibliographic record
Abstract
OBJECTIVE: To compare survival in Primary Progressive Aphasia to that in behavioral variant FTD (bvFTD) and FTD-ALS and to identify clinical, demographic and pathologic features impacting prognosis. BACKGROUND: PPA is a neurodegenerative syndrome targeting language networks with three major variants: semantic (PPA-S), logopenic (PPA-L) and nonfluent/agrammatic (PPA-G). While beginning as a focal aphasia, PPA eventually progresses to global dementia and ultimately death from disease complications. Although the natural history of PPA is recognized as variable, prognostic factors remain poorly understood. DESIGN/METHODS: We analyzed data from patients with clinical diagnoses of PPA, bvFTD, or FTD-ALS followed in our FTD Unit, including age of symptom onset, diagnosis and death or last observation. Pathologic diagnoses, where available, were dichotomized as primarily Tau (FTLD-Tau, PSP, CBD, AD) or non-Tau (FTLD-TDP-43). A cox regression analysis was performed to identify prognostic factors. RESULTS: Data from 110 patients were analyzed, including 26 PPA-G, 22 PPA-S, 18 PPA-L, 3 PPA-Other, 29 bvFTD and 12 FTD-ALS. Of these patients, 28 have died. Median survival of PPA patients from symptom onset (10 years) was longer than bvFTD (6 years) and FTD-ALS (6 years) (p<0.002). Survival in the three subtypes of PPA did not differ (9 years PPA-G, 11 years PPA-S, 10 years PPA-L). There was no effect of age of symptom onset or diagnosis, gender, handedness or years of education on survival. Neuropathology was available for 23 of the 28 patients who died. Of the19 patients with FTLD pathology, tauopathy was associated with longer survival than non-tauopathy (p<0.038). CONCLUSIONS: In our series of patients, PPA subjects had a longer survival from time of symptom onset when compared to bvFTD. Age, gender, handedness and years of education did not influence survival. A pathological diagnosis of tauopathy was associated with longer survival than non-tauopathy, consistent with previous literature. Funding: R21NS084156, R21NS077059, P50-AG005134
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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 | 0.001 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.001 |
| 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".