Table 1.
MAPT mutation carriers (n = 29) | GRN mutation carriers (n = 20) | C9orf72 mutation carriers (n = 31) | Non-carriers (n = 24) | p Value | Group differences | |
---|---|---|---|---|---|---|
Age at baseline, y | 52.6 ± 5.5 | 60.4 ± 7.4 | 62.1 ± 9.1 | 56.1 ± 5.7 | < 0.01 |
MAPT < GRN = C9orf72 NC < C9orf72 |
Sex (% female) | 10 (34.5%) | 12 (57.1%) | 13 (41.9%) | 11 (45.8%) | 0.6 | n.s |
Educational level a (median (IQR)) | 5 (2) | 5 (2) | 5 (2) | 5 (0) | 0.8 | n.s |
Disease duration, y | 1.4 ± 2.0 | 1.0 ± 1.1 | 3.1 ± 2.7 | NA | < 0.01 | MAPT = GRN < C9orf72 |
Subtype dis—apa—ster |
9—15—5 | 6—14—0 | 6—21—3 | NA | 0.3 | n.s |
MMSE | 25.9 ± 2.9 | 22.5 ± 6.3 | 26.5 ± 2.7 | 29.3 ± 0.8 | < 0.01 |
GRN < MAPT < NC GRN < C9orf72 |
FAB | 14.7 ± 3.2 | 10.0 ± 4.7 | 13.9 ± 3.4 | 16.1 ± 1.7 | < 0.01 | GRN < MAPT = NC |
Values indicate mean ± SD or n (%) unless otherwise specified
MAPT microtubule-associated protein tau, GRN progranulin, C9orf72 chromosome 9 open reading frame 72, NC non-carriers, dis disinhibited, apa apathetic, ster stereotypic, MMSE Mini-Mental State Examination, FAB Frontal Assessment Battery, n.s not significant
aVerhage Dutch educational system categorized into levels from 1 = less than 6 years of primary education to 7 = academic schooling