Table 2

Amyotrophic lateral sclerosis phenotypes. Overall and men versus women mean annual crude incidence rates (/100 000 population), 95% CIs and gender incidence rate ratios

PhenotypeOverall, incidence rate (95% CI)Men, incidence rate (95% CI)Women, incidence rate (95% CI)Men to women incidence rate ratio
Classic0.94 (0.85 to 1.04)1.17 (1.03 to 1.32)0.71 (0.61 to 0.83)1.65:1
Bulbar1.05 (0.96 to 1.15)1.04 (0.91 to 1.19)1.06 (0.94 to 1.20)0.98:1
Flail arm0.17 (0.13 to 0.21)0.28 (0.21 to 0.36)0.07 (0.04 to 0.12)4.00:1
Flail leg0.40 (0.34 to 0.47)0.40 (0.32 to 0.50)0.39 (0.31 to 0.48)1.03:1
Pyramidal0.28 (0.23 to 0.34)0.28 (0.21 to 0.36)0.27 (0.21 to 0.35)1.04:1
Respiratory0.03 (0.02 to 0.05)0.06 (0.03 to 0.10)0.01 (0 to 0.03)6.00:1
PLMN0.08 (0.06 to 0.11)0.11 (0.07 to 0.17)0.05 (0.03 to 0.08)2.04:1
PUMN0.12 (0.09 to 0.16)0.12 (0.08 to 0.18)0.12 (0.08 to 0.17)0.98:1
Overall ALS3.07 (2.89 to 3.25)3.46 (3.23 to 3.71)2.68 (2.44 to 2.90)1.29:1
  • ALS, amyotrophic lateral sclerosis; PLMN, pure lower motor neuron phenotype; PUMN, pure upper motor neuron phenotype.