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New Criteria for the Diagnosis of ALS

Introduction

Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease affecting the cortical upper motor neurons (UMN) and the lower motor neurons (LMN) in the brainstem and spinal cord. Disease pathophysiology remains incompletely understood since the first clinical-pathological correlations in the XIX century.1 Historically, the diagnosis of ALS is one of exclusion, requires clinical expertise and for decades relies on consensus diagnostic criteria including the El Escorial criteria, the revised El Escorial and the Awaji criteria.2-4 These criteria classify the diagnosis as possible, probable or definite ALS. The revised El Escorial criteria included EMG abnormalities in the assessment and added the “laboratory-supported, probable ALS” category. While initially conceptualized for research purposes, the El Escorial criteria are frequently utilized in clinical practice, but can add confusion regarding the communication of diagnostic certainty. Given the limited sensitivity and ambiguity of these diagnostic criteria, the World Federation of Neurology, the International Federation of Clinical Neurophysiology, the ALS Association, and the MND Association organized a panel meeting in Gold Coast, Australia, to propose simplified criteria for both clinical and research settings. The panel consensus is now known as the Gold Coast Criteria (GCC).5