Table III.
Characteristics of chromosome 9p-linked FTLD-ALS families.
| Reference | No. families (n patients) | FTLD or dementia | FTLD-ALS | ALS | Mean AAO (range) | Mean DD (range) | Molecular pathology (n patients) |
|---|---|---|---|---|---|---|---|
| Morita et al. 2006 | 1 (14) | 9 (64%) | 0 (0%) | 5 (36%) | 55.3 (45–64) | 4.3 (9) | NK (2) |
| Vance et al. 2006 | 1 (12) | 2 (17%) | 3 (25%) | 7 (58%) | 60.3 (39–72) | 3.0 (1–8) | Ub + (5) |
| Valdmanis et al. 2007 | 2 (16) | 3 (19%) | 3 (19%) | 10 (63%) | 55.1 (45–63) | 3.6 (9) | 0 |
| Luty et al. 2008 | 1 (11) | 7 (64%) | 2 (18%) | 2 (18%) | 52.9 (43–68) | 8.6 (1–18) | Ub +, TDP-43 +, FUS + (3) |
| Le Ber et al. 2009 | 6 (31) | 10 (32%) | 12 (39%) | 9 (29%) | 57.9 (41–84) | 3.6 (1–9) | Ub +, TDP-43 + (3) |
| Gijselinck et al. 2010 | 1 (9) | 8 (89%) | 0 (0%) | 1 (11%) | 58.1 (51–65) | 6.4 (1–17) | Ub +, TDP-43 + (1) |
| Boxer et al. 2010 | 1 (10) | 5 (50%) | 3 (30%) | 2 (20%) | 45.7 (35–57) | 5.8 (3–10) | Ub +, TDP-43 +, FUS- (3) |
| Pearson et al. 2010 | 1 (9) | 0 (0%) | 6 (67%) | 3 (33%) | 42.2 (31–52) | 3.6 (1–13) | Ub +, TDP-43 + (3) |
| All families | 12 (112) | 35 (31%) | 38 (34%) | 39 (35%) | 53.4 (31–84) | 4.9 (1–18) |
No. = number; AAO = age at onset; DD = disease duration; NK = not known.