A Study of the Clinical Characteristics and Severity of Amyotrophic Lateral Sclerosis from North India

Abstract

Introduction: Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease, and severity of disease at presentation decides prognosis and therapeutic options. We aimed to study the clinical characteristics and severity of ALS, and establish a correlation between King's ALS clinical staging and revised ALS Functional Rating Scale (ALSFRS-R) scores. Materials and Methods: All patients with clinically definite ALS presenting to the outpatients clinic of a tertiary care institute in North India were included. Their demographic and risk factor profiles were noted, and patients were categorized according to King's ALS clinical staging and ALSFRS-R scores. Correlations between the two scores were done using appropriate statistical tests. All data were analyzed using IBM SPSS version 26 for Macintosh. Results: Sixty-three patients with clinically definite ALS were included; of which 42 (66.7%) were male, with a sex ratio of 2:1. The mean duration of symptoms was 11.9 ± 6.6 months (range 3–24 months). Forty-five patients (71%) had limb-onset, and 18 (29%) had bulbar-onset ALS. The mean ALSFRS-R score was 36.5 ± 6.3 (range 16-48), and the scores were significantly lower in females (33.76 ± 5.60 vs. 37.81 ± 6.31; P = 0.01). On correlating the King's ALS clinical staging and ALSFRS-R scores, we found a score of >41 on ALSFRS-R was predictive of Stage 1, 34–41 of Stage 2, and 16–34 of Stage 3 disease. Conclusions: ALS has a male preponderance, but females present with more severe symptoms. There is a definite correlation between King's ALS clinical Stages 1-3 and ALSFRS-R scores.