1995–1997 (n = 231)2 (95% CI) | 2002–2004 (n = 234) (95% CI) | 1995–1997 and 2002–2004 (n = 465) (95% CI) | |
Median age (y) | 65.7 | 66.1 | 66.0 |
Sex (M:F) | 133:98 | 135:99 | 268:197 |
Limb:bulbar:generalised onset | 129:80:22 | 139:84:11 | 268:164:33 |
Family history of ALS | 9 | 20 | 29 |
Patients with FTD | 4† | 28 | 32 |
Mean diagnostic delay (months)‡ | 10.5 | 15.4 | 13.0 |
Median survival (months) | 17.2 | 16.1 | 16.4 |
Incidence per 100 000 py | 2.1 (1.8, 2.4) | 1.9* (1.5, 2.4) | 2.0 (1.9, 2.2) |
Incidence rate per 100 000 py over 15 y | 2.8 (2.4, 3.1) | 2.5* (2.2, 2.8) | 2.6 (2.4, 2.9) |
Female incidence per 100 000 py | 1.8 (1.4, 2.1) | 1.6 (1.3, 2.0) | 1.7 (1.5, 2.0) |
Male incidence per 100 000 py | 2.5 (2.0, 2.9) | 2.3 (1.9, 2.7) | 2.4 (2.1, 2.7) |
Bulbar incidence per 100 000 py | 0.9 (0.8, 1.1) | 0.8 (0.6, 1.0) | 0.9 (0.7, 1.0) |
Limb incidence per 100 000 py | 1.2 (1.0, 1.4) | 1.2 (1.0, 1.4) | 1.2 (1.0, 1.3) |
*Age and gender adjusted to the 1996 Irish population.
†The Irish ALS Register did not routinely collect cognitive data until 2000.
‡The t test statistic = −3.2, p = 0.001.
ALS, amyotrophic lateral sclerosis; FTD, frontotemporal dementia; py, person-years.