Abstract
Background Amyotrophic lateral sclerosis (ALS) is a fatal disease whose diagnosis and early management can improve survival. The most used diagnostic criteria are the revised El Escorial criteria (rEEC) and Awaji criteria (AC). The comparison of their sensitivities showed contradictory results. Our study aimed to compare the sensitivities of these two criteria in the diagnosis of definite ALS, at first visit, in a Tunisian hospital cohort. Materials and Methods This was a retrospective study including 173 patients diagnosed with ALS at the Department of Neurology of the Razi Hospital between January 2003 and April 2018.After studying the clinical features of the disease in our study population,each patient was categorized according to the rEEC and AC based on data collected in his medical record during his first visit to our department. Then, we compared the sensitivities of these two criteria in the diagnosis of definite ALS. Results Our Tunisian cohort was characterized by a slower disease progression. The sensitivity of the AC (69.4%) was significantly higher than that of the rEEC (40.5%) (p < 0.001). When the clinical signs evolved for less than 6 months, the sensitivities were 61% for AC and 12% for rEEC (p < 0.001). After 24 months of disease progression, the sensitivities were 78.2% for AC and 69.1% for rEEC (p = 0.063). It was impossible to categorize seventeen patients by the two criteria. Conclusion Our study demonstrated that patients in AC are more sensitive than rEEC in the early diagnosis of ALS in our Tunisian cohort. However, this superiority is gradually reduced during the evolution of the disease.
Highlights
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease defined by the association of upper motor neuron (UMN) and lower motor neuron (LMN) signs in the bulbar and spinal territories, which typically leads to death with a median survival of 36 months [1]
These criteria have been considered insufficient by several authors [5, 6] because of the low diagnostic sensitivity and the lack of importance to electromyography (EMG) signs, the fasciculation potentials recorded during EMG examination
Electrophysiological, biology, and imagery (cerebral and/or medullary magnetic resonance imaging (MRI)) data, to exclude potential ALS mimic disorders. e demographic, clinical, and first EMG data of those patients have been collected from their medical record. e juvenile form of ALS corresponds to cases where the age of onset was
Summary
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease defined by the association of upper motor neuron (UMN) and lower motor neuron (LMN) signs in the bulbar and spinal territories, which typically leads to death with a median survival of 36 months [1].Early treatment can improve survival, thanks to a few drugs including riluzole and more recently edaravone, which has been approved since 2017 [2].Sometimes the early diagnosis of ALS is still difficult because there is no specific diagnostic test or biomarker for the disease and because of the heterogeneity of the phenotypes.is difficulty led to the organization of the El Escorial Consensus conference in 1991 and 7 years later that of Airlie House, which, respectively, proposed the El Escorial criteria [3] and revised El Escorial criteria (rEEC) [4] to harmonize protocols and therapeutic trials and provide as early as possible the proper care to patients.these criteria have been considered insufficient by several authors [5, 6] because of the low diagnostic sensitivity and the lack of importance to electromyography (EMG) signs, the fasciculation potentials recorded during EMG examination.us, the Awaji criteria (AC) were proposed in 2008 [7], which accepted the existence of LMN involvement as wellNeurology Research International based on the presence of clinical signs as on the presence of EMG signs including fasciculation potential.Since many studies have compared the diagnostic sensitivities of AC and rEEC, with contradictory results [8,9,10,11,12,13], which could be related to the differences between the study populations and methodological limitations.e aim of the present study was to compare the sensitivities of AC with rEEC in the diagnosis of definite ALS, at first visit, in a Tunisian hospital cohort. Is difficulty led to the organization of the El Escorial Consensus conference in 1991 and 7 years later that of Airlie House, which, respectively, proposed the El Escorial criteria [3] and revised El Escorial criteria (rEEC) [4] to harmonize protocols and therapeutic trials and provide as early as possible the proper care to patients These criteria have been considered insufficient by several authors [5, 6] because of the low diagnostic sensitivity and the lack of importance to electromyography (EMG) signs, the fasciculation potentials recorded during EMG examination. Our study aimed to compare the sensitivities of these two criteria in the diagnosis of definite ALS, at first visit, in a Tunisian hospital cohort. This superiority is gradually reduced during the evolution of the disease
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