Diagnostic dilemmas in amyotrophic lateral sclerosis

The El Escorial criteria for the diagnosis of amyotrophic lateral sclerosis (ALS) were introduced in 1990 to provide a standardised and diagnostically reliable approach to the early recognition of this disease. It is still un clear, however, whether these are the best criteria available for the early diagnosis of ALS. We applied these criteria retrospectively to clinical ma terial available from 34 cases of autopsy proven ALS in an attempt to valid ate their usefulness in a pathological series. The clinical material was re viewed and specific clinical signs and anatomical levels of involvement rec orded. Only signs listed in the criteria were included in the analysis. The El Escorial criteria for clinically definite ALS demonstrated high sensiti vity and accuracy when validated in this clinicopathological study. Neuroim aging and electromyography were important in shortening the time in which p atients were given a diagnosis of clinically definite ALS. Other diagnostic dilemmas in ALS and the role of early diagnosis of this disease are discus sed. (C) 1999 Published by Elsevier Science B.V. All rights reserved.