Awaji ALS criteria increase the diagnostic sensitivity in patients with bulbar onset

ObjectiveTo assess whether Awaji criteria improve the sensitivity of diagnosis for amyotrophic lateral sclerosis (ALS). In Awaji ALS criteria, fasciculation potentials are regarded as evidence of acute denervation in the presence of chronic neurogenic changes on needle electromyography.MethodsWe reviewed clinical and neurophysiological data of 113 consecutive patients who were suspected as suffering ALS. The six muscles (trapezius, biceps, first dorsal interosseous, T10-paraspinalis, vastus lateralis, and tibialis anterior muscles) were examined by EMG, focusing on the presence of fasciculation potentials. The sensitivity of revised El Escorial (R-EEC) and Awaji criteria was compared.ResultsProbable or definite ALS was diagnosed in 61% of the patients by R-EEC and 71% by Awaji criteria. By applying Awaji criteria; (1) 17 of the 44 patients categorized as possible ALS by R-EEC reached to probable/definite ALS, 11 of whom had bulbar onset, (2) in 48 patients with bulbar onset, the proportion of probable/definite ALS increased from 59% to 82%, (3) in 62 patients with limb onset, the proportion of probable/definite ALS was 61% (63% by R-EEC).ConclusionsAwaji criteria improve the sensitivity of ALS diagnosis in patients with bulbar onset, but not in those with limb onset.SignificanceAccepting fasciculation potentials as evidence of acute denervation increases the diagnostic sensitivity of ALS, particularly in patients with bulbar onset, and contributes to early diagnosis.

► Awaji ALS criteria have recently been proposed to increase the diagnostic sensitivity with regarding fasciculation potentials as evidence of acute denervation. ► We studied the utility of Awaji criteria in 113 consecutive ALS patients, and compared results with conventional criteria. ► By applying Awaji criteria, the sensitivity significantly increased in ALS patients with bulbar onset, suggesting the importance of fasciculation potentials in the diagnosis of ALS.