Table 1 Demographics and clinical features of Irish patients diagnosed with amyotrophic lateral sclerosis in the 1995–1997 and 2002–2004 cohorts
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)65.766.166.0
Sex (M:F)133:98135:99268:197
Limb:bulbar:generalised onset129:80:22139:84:11268:164:33
Family history of ALS92029
Patients with FTD4†2832
Mean diagnostic delay (months)‡10.515.413.0
Median survival (months)17.216.116.4
Incidence per 100 000 py2.1 (1.8, 2.4)1.9* (1.5, 2.4)2.0 (1.9, 2.2)
Incidence rate per 100 000 py over 15 y2.8 (2.4, 3.1)2.5* (2.2, 2.8)2.6 (2.4, 2.9)
Female incidence per 100 000 py1.8 (1.4, 2.1)1.6 (1.3, 2.0)1.7 (1.5, 2.0)
Male incidence per 100 000 py2.5 (2.0, 2.9)2.3 (1.9, 2.7)2.4 (2.1, 2.7)
Bulbar incidence per 100 000 py0.9 (0.8, 1.1)0.8 (0.6, 1.0)0.9 (0.7, 1.0)
Limb incidence per 100 000 py1.2 (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.