Table 3

HRs for El-Escorial criteria after inclusion in final model

El-Escorial categoryHR95% CIWald test
Suspected0.860.68 to 1.080.199
Probable—laboratory supported1.381.31 to 1.44<0.001
Probable1.491.39 to 1.60<0.001
Definite2.091.99 to 2.21<0.001
Sex/site of onset/C9orf72
 Spinal onset excluding male C9orf72 expanded cases1
 All bulbar onset cases1.331.21 to 1.45<0.001
 Male C9orf72 expanded cases only1.571.26 to 1.96<0.001
  • HRs for each of the El-Escorial criteria from a Royston-Parmar model on the hazard scale with 3 degrees of freedom and including age of onset (time varying), diagnostic delay, site of onset, country and sex-site-C9 as a hybridised variable with a variance–covariance matrix allowed to pool by country. Note that Belgium omitted as the El-Escorial criteria was not available, the UK data included only Probable and Definite category ALS, while Ireland does not use the ‘probable laboratory supported’. Nevertheless, HRs are in line with expectations showing a gradually increasing hazard with increasing category severity.