Table 1.
Demographics of included brain donors
| All donors | FTLD-TDP | FTLD-tau | FTLD-FUS | P-value | |
|---|---|---|---|---|---|
| n (%) | 87 (100%) | 46/87 (53%) | 34/87 (39%) | 7/87 (8%) | — |
| Gender (M:F) | 46:41 | 21:25 | 19:15 | 6:1 | 0.13a |
| Age at onset (years; mean ± SD) | 57.6 ± 10.7 | 59.4 ± 9.4 | 56.9 ± 11.5 | 48.6 ± 11.4 | 0.04b,* (FTLD-TDP versus FTLD-FUS 0.03*) |
| Age at death (years; mean ± SD) | 66.0 ± 9.7 | 67.4 ± 8.0 | 66.1 ± 10.6 | 56.1 ± 11.4 | 0.02b,* (FTLD-TDP versus FTLD-FUS 0.01*; FTLD-tau versus FTLD-FUS 0.03*) |
| Disease duration (years; mean ± SD) | 8.4 ± 5.0 | 8.0 ± 4.7 | 9.2 ± 5.2 | 7.6 ± 6.5 | 0.54 |
| Clinical diagnosis (%) | 61/87 (70%) bvFTD; 21/87 (24%) PPA; 1/87 (1%) PSP; 4/87 (5%) ALS-FTD | 28/46 (61%) bvFTD; 14/46 (30%) PPA; 4/46 (9%) ALS-FTD | 27/34 (79%) bvFTD; 6/34 (18%) PPA; 1/34 (3%) PSP | 5/7 (72%) bvFTD; 1/7 (14%) PPA; 1/7 (14%) CBS | — |
| Genetic status (%) | 15/87 (17%) C9orf72; 11/87 (13%) MAPT; 6/87 (7%) GRN; 1/87 (1%) TARDBP | 15/46 (33%) C9orf72; 6/46 (13%) GRN; 1/46 (2%) TARDBP | 11/34 (32%) MAPT | — | |
| Brain weight (g; mean ± SD) | 1061.6 ± 166.7 | 1053.4 ± 160.7 | 1052.1 ± 180.1 | 1161.7 ± 117.1 | 0.26b |
| Pathological subgroups (% main pathology) | — | 14/46 (31%) TDP-A; 13/46 (28%) TDP-B; 11/46 (24%) TDP-C; 6/46 (13%) TDP-E; 2/46 (4%) TDP-U | 20/34 (59%) PiD; 3 (9%) CBD; 4 (12%) PSP; 7 (20%) tauopathy-U | 5/7 (72%) aFTLD-U; 2/7 (28%) NIFID | — |
| Thal stage for amyloid-β (median ± IQR) | 1 (0–2) | 1 (0–3) | 0 (0–1) | 0 (0–0.5) | 0.01c,* (FTLD-TDP versus FTLD-tau 0.04*) |
| Braak stage for tau (median ± IQR) | 2 (0–2) | 2 (0.5–2) | NA | 1 (0–2.25) | NA |
| Braak stage for Lewy body pathology (median ± IQR) | 0 (0–0) | 0 (0–0) | 0 (0–0) | 0 (0–0) | 0.07c |
Values are expressed as %, mean ± standard deviation (SD), or median ± interquartile range (IQR). ADL = activities of daily living; ALS = amyotrophic lateral sclerosis; bvFTD = behavioural variant frontotemporal dementia; CBS = corticobasal syndrome; U = unknown (did not meet criteria for any specific subtype); aFTLD-U = atypical FTLD with ubiquitin inclusions; NIFID = neuronal intermediate filament inclusion disease; PPA = primary progressive aphasia.
Pearson’s chi-squared test.
One-way ANOVA (Tukey's post hoc test).
Kruskal–Wallis one-way ANOVA and pairwise comparisons with Bonferroni correction.
P < 0.05.