Table 2.
Demographic and Clinical Characteristics of Probands for the Original, Replication, and Combined Cohorts by Categorya
| Characteristic | Original Cohort |
Replication Cohort |
Combined Cohort | ||||
|---|---|---|---|---|---|---|---|
| High | Medium | Low | Apparent Sporadic |
Unknown Significance |
|||
| No. of males/females | 106/88 | 63/49 | 19/20 | 16/15 | 30/16 | 50/41 | 54/45 |
| Age at onset, median (range), y | 59 (30–80) | 60 (33–80) | 56 (31–74) | 57 (33–71) | 61 (37–79) | 58 (30–80) | 61 (38–80) |
| Ethnicity, No. | |||||||
| White | 185 | 106 | 38 | 29 | 41 | 88 | 95 |
| Race other than white | 9 | 6 | 1 | 2 | 5 | 3 | 4 |
| FTLD clinical diagnosis, No. | |||||||
| bvFTD | 81 | 34 | 15 | 12 | 20 | 36 | 32 |
| SD | 21 | 16 | 5 | 5 | 3 | 10 | 14 |
| PNFA | 11 | 10 | 2 | 1 | 4 | 2 | 12 |
| LPA | 13 | 4 | 1 | 3 | 2 | 4 | 7 |
| CBS | 46 | 21 | 10 | 9 | 6 | 23 | 19 |
| PSP | 8 | 16 | 1 | 1 | 4 | 9 | 9 |
| FTLD with ALS | 14 | 11 | 5 | 0 | 7 | 7 | 6 |
Abbreviations: ALS, amyotrophic lateral sclerosis; bvFTD, behavioral variant frontotemporal dementia; CBS, corticobasal syndrome; FTLD, frontotemporal lobar degeneration; LPA, logopenic progressive aphasia; PNFA, progressive nonfluent aphasia; PSP, progressive supranuclear palsy; SD, semantic dementia.
a
Demographic characteristics of original cohort and replication cohorts were not significantly different.