Age-related reference values for the pediatric Scale for Assessment and Rating of Ataxia: a multicentre study

Tjitske F Lawerman, Rick Brandsma, Huibert Burger, Johannes G M Burgerhof, Deborah A Sival, the Childhood Ataxia and Cerebellar Group of the European Pediatric Neurology Society

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Abstract

AimFor reliable assessment of ataxia severity in children, the Childhood Ataxia and Cerebellar Group of the European Pediatric Neurology Society aimed to validate the Scale for Assessment and Rating of Ataxia (SARA) according to age.

MethodTwenty-two pediatric ataxia experts from 15 international institutions scored videotaped SARA performances in 156 typically developing children (4-16y: m/f=1; 12 children per year of age; including nine different nationalities). We determined age-dependency and reliability of pediatric SARA scores by a mixed model.

ResultsIn typically developing children, age was the only variable that revealed a relationship with SARA scores (p

InterpretationIn typically developing European children, both SARA scores and interobserver agreement are age-dependent. For reliable interpretation of pediatric SARA scores, consideration of the underlying test construct appears prudent. These data will hopefully contribute to a correct and uniform interpretation of longitudinal SARA scores from childhood to adulthood.

Original languageEnglish
Pages (from-to)1077-1082
Number of pages6
JournalDevelopmental Medicine and Child Neurology
Volume59
Issue number10
Early online date17-Aug-2017
DOIs
Publication statusPublished - Oct-2017

Keywords

  • HEREDITARY CEREBELLAR-ATAXIA
  • PLACEBO-CONTROLLED TRIAL
  • IDEBENONE TREATMENT
  • FRIEDREICHS-ATAXIA
  • DOUBLE-BLIND
  • FOLLOW-UP
  • CHILDREN
  • RILUZOLE

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