The Criteria for Diagnosing Amyotrophic Lateral Sclerosis May Be Unsuitable for Clinical Use

Background: The existing diagnostic criteria for definite, probable and possible amyotrophic lateral sclerosis (ALS) require at least one upper motor neuron (UMN) sign. However, we think some cases of ALS or motor neuron disease may be not able to fulfill the required criteria. We investigated whether the clinical presentation and course of patients with ALS or motor neuron disease fulfill the required criteria or not. Methods: In total, 296 patients who were diagnosed with ALS or motor neuron disease in the institute in the past 15 years were retrospectively investigated and analyzed. Results: In total, 108 patients (36.5%) who exhibited lower motor neuron (LMN) signs but not UMN signs at the early stage of the disease were diagnosed with ALS or motor neuron disease. Sixty-four of these 108 patients (59.2%) who developed respiratory failure and swallowing difficulty or UMN signs during the 5-year follow-up period were diagnosed with clinical ALS. No significant difference in the survival probability was observed between patients who exhibited both UMN and LMN signs and patients who exhibited only LMN signs. Conclusion: These findings suggest that some of motor neuron disease cases who only exhibit LMN signs can be diagnosed as ALS. I suggest the revised El Escorial Criteria for the Diagnosis of ALS may be unsuitable for clinical use. J Neurol Res. 2016;6(4):57-64 doi: http://dx.doi.org/10.14740/jnr383w