Table 1.
Cohort demographics
| Cohort 1 | ||||
|
NC (n = 8) |
PMC (n = 16) |
PPA (n = 13) |
bvFTD (n = 16) |
|
| Age, median years (range)a | 52 (24–65) | 53 (31–71) | 65 (52–79) | 61 (40–78) |
| Female, N (%) | 4 (50) | 10 (62) | 8 (57) | 6 (37) |
| Age at onset, median years (range) | – | – | 63 (50–78) | 59 (39–77) |
| Years to expected onsetb, median years (range) | – | 8 (−24,+ 3) | – | – |
| Mutation, N (%) | ||||
| C9orf72 | – | 8 (50) | 0 | 2 (13) |
| GRN | – | 8 (50) | 1 (8) | – |
| VCP | – | – | – | 1 (6) |
| Cohort 2 |
Control (n = 18) |
FTDc (n = 13) |
AD (n = 79) |
|
| Age, median years (range)d | 81 (74–86) | 68 (50–83) | 72 (54–88) | |
| Female, N (%) | 10 (56) | 4 (31) | 49 (62) | |
NC Non carriers, PMC Presymptomatic mutation carriers, PPA Primary progressive aphasia, bvFTD – behavioural variant FTD, AD Alzheimer’s disease
a Differences in age were found between PPA and unaffected individuals (ANOVA, p = 0.001, pairwise post hoc test PPA vs NC, p = 0.01, PPA vs PMC, p = 0.01). Differences where found between FTD as a whole group and unaffected individuals (t-test, p < 0.001)
b Difference between the subjects age at sampling and the mean age at onset in their family
c Clinical phenotype: 7 bvFTD and 5 SD
d Differences in age were found between controls and FTD/AD (ANOVA, p < 0.001, pairwise post hoc test controls vs FTD, p < 0.001, controls vs AD, p < 0.001)