title: Assessment of speech in early-onset ataxia: a pilot study.
abstract: OBJECTIVE
  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).
  
  
  METHODS
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Marieke J
authors.initials: MJ
authors.lastname: Kuiper
authors.affiliation: Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Rick
authors.initials: R
authors.lastname: Brandsma
authors.affiliation: Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Tjitske F
authors.initials: TF
authors.lastname: Lawerman
authors.affiliation: Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Roelineke J
authors.initials: RJ
authors.lastname: Lunsing
authors.affiliation: Ear, Nose and Throat Department, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Anne L
authors.initials: AL
authors.lastname: Keegstra
authors.affiliation: Department of General Practice, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Huibert
authors.initials: H
authors.lastname: Burger
authors.affiliation: Department of Paediatrics, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Tom J
authors.initials: TJ
authors.lastname: De Koning
authors.affiliation: Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Marina A J
authors.initials: MAJ
authors.lastname: Tijssen
authors.affiliation: Department of Paediatrics, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Deborah A
authors.initials: DA
authors.lastname: Sival
mesh_terms: D000293:Adolescent; D000328:Adult; D001259:Ataxia / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D010372:Pediatrics; D010865:Pilot Projects; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D013064:Speech Disorders / Q000175:diagnosis* / Q000209:etiology*; D013223:Statistics as Topic; D055815:Young Adult