Differences in Initial Symptoms between Amyotrophic Lateral Sclerosis (ALS) and Primary Lateral Sclerosis (PLS) in a Referral Centre

RODRIGUEZ G; GARGIULO MONACHELLI, G.M; GONZÁLEZ DENISELLE M. CLAUDIA; DE NICOLA A. F.; SICA REP

Seattle, USA

Congreso; 61st Annual Meeting of the American Academy of Neurology; 2009

American Academy of Neurology

INTRODUCTION: ALS and PLS are neurodegenerative diseases with motoneuron compromise. In the former, both upper and lower motoneurons are involved, and in the latter, only the upper motoneuron degenerates. OBJECTIVES: The purpose of this study was to determine epidemiologic differences between ALS and PLS. METHODOLOGY: We reviewed the Motoneuron Disease Section database in the Ramos Mejia Hospital for patients with ALS according to the El Escorial criteria, and for patients with PLS according to criteria published by Pringle et al. We gathered information on when the disease started, which was the first symptom and when was the diagnosis made. Statistical analysis for parametric variables was conducted using the Student’s T-test, and for non parametric the Chi-square (X2). RESULTS: We found 166 ALS patients and 14 PLS patients. In the ALS group 65 were women (39.2%) and 101 were men (60.8%). In the PLS group 10 were women (71.4%) and 4 were men (28.6%) (X2 p = 0.018). The mean age in ALS was 55.49 years and in PLS 55.64 years. We observed a four-fold increase in the mean time from onset to diagnosis in PLS patients compared to ALS (80.64 months vs 20 months p < 0.0001). The most frequent initial symptom for both diseases was weakness in one extremity (ALS 50.9% and PLS 28.6%). We also registered weakness in 2 extremities and unstable gait more often in PLS than ALS patients (14.3% vs 5.5% and 14.3% vs 0.6% respectively). CONCLUSION: We found more women among PLS patients (71.4%) than ALS patients (39.2%). The time onset to diagnosis was longer in PLS patients probably due to its more benign course and lower incidence. Weakness in 2 extremities and unstable gait were more frequently observed as initial symptoms in PLS patients.