Table 1. Demographic Features and Goldman Scores in Frontotemporal Lobar Degeneration Subtypes.
| Feature | Incident cases | ||||
|---|---|---|---|---|---|
| BVFTD (n = 107) | PPA (n = 76) | CBS or PSP (n = 69) | FTD-ALS (n = 15) | Total (N = 267) | |
| Age, mean (SD), y | 64.19 (10.10) | 67.12 (7.63) | 70.38 (7.32) | 65.53 (9.10) | 66.70 (9.02) |
| Sex, No. (%) | |||||
| Female | 45 (42.06) | 31 (40.79) | 30 (43.48) | 5 (33.33) | 111 (41.57) |
| Male | 62 (57.94) | 45 (59.21) | 39 (56.52) | 10 (66.67) | 156 (58.43) |
| Educational level, y | |||||
| Missing data, No. (%) | 9 (8.41) | 4 (5.26) | 10 (14.49) | 1 (6.67) | 24 (8.99) |
| Mean (SD) | 11.50 (3.74) | 11.93 (3.31) | 10.64 (4.18) | 12.00 (3.88) | 11.45 (3.75) |
| Disease duration, mean (SD), mo | 36.89 (21.53) | 33.41 (19.38) | 33.23 (19.77) | 20.93 (15.04) | 34.06 (20.39) |
| AD ruled out, No. (%)a | 49 (45.79) | 28 (36.84) | 15 (21.74)b | 7 (46.67) | 99 (37.08) |
| Goldman score, No. (%) | |||||
| 1 | 7 (6.54) | 1 (1.32) | 1 (1.45) | 1 (6.67) | 10 (3.75) |
| 2 | 9 (8.41) | 3 (3.95) | 1 (1.45) | 1 (6.67) | 14 (5.24) |
| 3 | 17 (15.89) | 5 (6.58) | 6 (8.70) | 0 (0.00) | 28 (10.49) |
| 3.5 | 14 (13.08) | 18 (23.68) | 10 (14.49) | 1 (6.67) | 43 (16.10) |
| 4 | 60 (56.07) | 49 (64.47) | 51 (73.91) | 12 (80.00) | 172 (64.42) |
| CDR plus NACC FTLD sum of boxes | |||||
| Missing, No. (%) | 15 (14.02) | 7 (9.21) | 7 (10.14) | 3 (20.00) | 32 (11.99) |
| Median (range) | 6.0 (1.0-20.0) | 3.0 (0.5-21.0) | 3.5 (0.5-23.0) | 4.5 (1.0-20.0) | 4.0 (0.5-23.0) |
Abbreviations: AD, Alzheimer disease; BVFTD, behavioral variant of frontotemporal dementia; CBS, corticobasal syndrome; CDR plus NACC FTLD, Clinical Dementia Rating Dementia Staging Instrument plus behavior and language domains from the National Alzheimer Coordinating Centre and Frontotemporal Lobar Degeneration modules; FTD-ALS, frontotemporal dementia with amyotrophic lateral sclerosis; PPA, primary progressive aphasia; PSP, progressive supranuclear palsy.
Ruled out by either cerebrospinal fluid markers (amyloid-β, tau, and P-tau), positron emission tomography image negative for amyloid, demonstration of FTLD-related pathogenetic sequence variations, or following autopsy.
AD ruled out for 11 of the 27 CBS cases (40.74%) and for 4 of the 42 PSP cases (9.52%).