Diagnostic criteria for amyotrophic lateral sclerosis: a multicentre study of inter-rater variation and sensitivity

Diagnostic criteria for amyotrophic lateral sclerosis (ALS) have been developed to standardise diagnosis and patient recruitment in trials. A research group of the World Federation of Neurology (WFN) devised the "El Escorial criteria" (EEC) (Brooks et al., 1994), which were later modified to increase their diagnostic sensitivity as the "revised El Escorial Criteria" (rEEC) (Brooks et al., 2000) with the objective of encouraging earlier inclusion in trials (Ross et al., 1998). The rEEC have been found to have high specificity, but to lack sensitivity, thus failing to promote earlier patient inclusion in clinical trials (Traynor et al., 2000; Zoccolella et al., 2006; Turner et al., 2009).

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