Abstract

To evaluate speech intelligibility and dysarthria, correlated to the functional assessment of Amyotrophic Lateral Sclerosis (ALS). Quantitative-descriptive study approved by REC under No. CAAE 62912416.4.0000.5404, comprised of 19 individuals with sporadic or familiar ALS. Data were collected using the Dysarthria Protocol and the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-Re). We used visual analogue scale (VAS) to assess speech intelligibility and summary measures; and Spearman's coefficients of correlation for the instruments with significance level of 5%. Speech intelligibility is compromised (41.37±39.73) in varied degrees with positive correlation with the general degree of dysarthria (p=<.0001), and with all the analyzed speech parameters, indicating impact on the speech deterioration of the studied group. There is negative correlation between speech intelligibility and the results of the bulbar sections - speech and deglutition (p=0.0166), arm - activities with the upper limb (p=0.0064) and leg - activities with the lower limb (p=0.0391). Breathing (p=0.0178), phonation (p=0.0334) and resonance (p=0.0053) parameters showed a negative correlation with the item "speech" of the ALSFRS-Re. Results show impaired speech intelligibility and dysarthria, and evidence breathing, phonation and resonance as important markers of the disease progression. A thorough and early evaluation of the oral motor production allows for a better management of alterations in ALS.

Highlights

  • Amyotrophic Lateral Sclerosis (ALS) is a heterogeneous neurodegenerative disease, which presents motor, cognitive, and behavioral changes

  • The results show that speech intelligibility is compromised (41.37 ± 39.73) in people with ALS studied and this aspect is influenced by all the components analyzed

  • The findings indicate a positive correlation between speech intelligibility and dysarthric changes in the population studied, which negatively influence the communication functionality of the person with ALS

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Summary

Introduction

Amyotrophic Lateral Sclerosis (ALS) is a heterogeneous neurodegenerative disease, which presents motor, cognitive, and behavioral changes. Recent pathophysiological studies show genetic, environmental, and lifestyle causes as the main causes of the disease[1]. 90% of cases appear sporadically and approximately 10% are of a family nature. There are no specific disease biomarkers reported in the literature, previous research reported a relationship with findings of ALS-related gene mutations and reduced dismutation activity of the superoxide dismutase enzyme SOD1(2). A recent study indicated that skeletal muscle microRNAs (miRNAs), MiR-424, and miR206 are potential prognostic markers for ALS[3]. No significant environmental factors are reported; smoking and lean body structure can be some aggravating factors[2]

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