Assessment of speech in early-onset ataxia: a pilot study

Authors

  • Marieke J Kuiper,

    1. Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
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  • Rick Brandsma,

    1. Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
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  • Tjitske F Lawerman,

    1. Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
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  • Roelineke J Lunsing,

    1. Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
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  • Anne L Keegstra,

    1. Ear, Nose and Throat Department, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
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  • Huibert Burger,

    1. Department of General Practice, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
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  • Tom J De Koning,

    1. Department of Paediatrics, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
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  • Marina A J Tijssen,

    1. Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
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  • Deborah A Sival

    Corresponding author
    1. Department of Paediatrics, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
    • Correspondence to Deborah A Sival at Department of Paediatrics, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, PO Box 30001, 9700 RB Groningen, the Netherlands. E-mail: d.a.sival@umcg.nl

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Abstract

Aim

The aim of the study was to determine whether paediatric ataxia speech subscores are reliably applicable for international early-onset ataxia (EOA) databases. If so, we reasoned that ataxia speech subscores should be associated with ataxia scores and involve high interobserver agreement, including those for internationally applicable Scale for Assessment and Rating of Ataxia (SARA) syllable repetition tasks (SARASRT).

Method

Three independent paediatric neurologists and a speech therapist scored speech in 52 healthy children (mean age 10y, range 4–16y) and 40 individuals with EOA (mean age 15y, range 5–34y). We compared ataxia speech subscores for the association with age and ataxia scores as well as interobserver reliability.

Results

In healthy children, ataxia speech subscores were moderately associated with age (International Cooperative Ataxia Rating Scale [ICARS]: r=−0.515; SARA: r=−0.321; p<0.05) and with ataxia scores (ICARS: r=0.552; SARA: r=0.336; p<0.05), and revealed slight to moderate interobserver agreement (ICARS-intraclass correlation coefficient [ICC]: 0.380; SARA-ICC: 0.185; SARASRT-ICC: 0.509). In EOA, speech subscores have a strong association with ataxia scores (ICARS: r=0.735; SARA: r=0.730; p<0.001) and revealed substantial to nearly perfect interobserver agreement (ICARS-ICC: 0.812; SARA-ICC: 0.854; SARASRT-ICC: 0.724).

Interpretation

Early-onset ataxia speech subscores are associated with ataxia and also reveal high interobserver agreement, including those internationally applicable to SARASRT. We conclude that SARASRT appears to be applicable for EOA databases. However, before syllable repetition tasks are included, we would advise to wait for the results published by the international Childhood Ataxia and Cerebellar Group.

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