Modern Diagnosis of Amyotrophic Lateral Sclerosis - an Update of the Diagnostic Criteria

The diagnosis of amyotrophic lateral sclerosis (ALS) is still challenging for clinicians. In the absence of a reliable biomarker, the clinical and electromyographic examination is of major importance to diagnose ALS. Mainly developed for clinical trials, the well-established revised El-Escorial criteria and Awaji criteria are sometimes also used in clinical routine. However, both diagnostic criteria are complex and disconcerting especially for patients because of various categories like "possible ALS" and "probable ALS". Additionally, a definite diagnosis can only be made late in the course of the disease, or even sometimes it is not possible to fulfill the diagnostic criteria. The newly proposed Gold Coast criteria are a simplified synopsis of the previous diagnostic criteria. The combination of progressive symptoms, clinical or electromyographic evidence of lower and upper motor neuron dysfunction or progressive lower motor neuron dysfunction, and the exclusion of other diseases lead to the diagnosis of ALS.