Abstract

BackgroundAmyotrophic lateral sclerosis (ALS) is a fatal degenerative disease of a rapid course. In 25% of ALS sufferers, speech disorders occur as prodromal symptoms of the disease. Impaired communication affects physical health and has a negative impact on mental and emotional condition. In this study, we assessed which domains of speech are particularly affected in ALS. Subsequently, we estimated possible correlations between the ALS patients’ subjective perception of their speech quality and an objective assessment of the speech organs carried out by an expert.MethodsThe study group consisted of 63 patients with sporadic ALS. The patients were examined for articulatory functions by means of Voice Handicap Index (VHI) and the Frenchay Dysarthria Assessment (FDA).ResultsOn the basis of the VHI scores, the entire cohort was divided into 2 groups: group I (40 subjects) with mild speech impairment, and group II (23 subjects) displaying moderate and profound speech deficits. In an early phase of ALS, changes were typically reported in the tongue, lips and soft palate. The FDA and VHI-based measurements revealed a high, positive correlation between the objective and subjective evaluation of articulation quality.ConclusionsDeterioration of the articulatory organs resulted in the reduction of social, physical and emotional functioning. The highly positive correlation between the VHI and FDA scales seems to indicate that the VHI questionnaire may be a reliable, self-contained tool for monitoring the course and progression of speech disorders in ALS.Trial registrationNCT02193893.

Highlights

  • Amyotrophic lateral sclerosis (ALS) is a fatal degenerative disease of a rapid course

  • Deterioration of the articulatory organs resulted in the reduction of social, physical and emotional functioning

  • The highly positive correlation between the Voice Handicap Index (VHI) and Frenchay Dysarthria Assessment (FDA) scales seems to indicate that the VHI questionnaire may be a reliable, self-contained tool for monitoring the course and progression of speech disorders in ALS

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Summary

Introduction

Amyotrophic lateral sclerosis (ALS) is a fatal degenerative disease of a rapid course. In 25% of ALS sufferers, speech disorders occur as prodromal symptoms of the disease. We assessed which domains of speech are affected in ALS. We estimated possible correlations between the ALS patients’ subjective perception of their speech quality and an objective assessment of the speech organs carried out by an expert. Amyotrophic lateral sclerosis (ALS) is a rapidly progressing and fatal neurological disease characterized by gradual degeneration of the upper and lower motor neurons. The etiology of ALS is multifactorial, affecting both neurons and glial cells [1]. Typical symptom of disease progression is bulbar dysarthria, which is the commonest form of speech impairment in ALS. In 25% of ALS patients speech deterioration is the first symptom of the disease [5]

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