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

作者：Kuiper Marieke J; Brandsma Rick; Lawerman Tjitske F; Lunsing Roelineke J; Keegstra Anne L; Burger Huibert; De Koning Tom J; Tijssen Marina A J; Sival Deborah A^*

来源：Developmental Medicine and Child Neurology, 2014, 56(12): 1202-1206.

DOI：10.1111/dmcn.12517

AimThe 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). %26lt;br%26gt;MethodThree 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. %26lt;br%26gt;ResultsIn healthy children, ataxia speech subscores were moderately associated with age (International Cooperative Ataxia Rating Scale [ICARS]: r=-0.515; SARA: r=-0.321; p%26lt;0.05) and with ataxia scores (ICARS: r=0.552; SARA: r=0.336; p%26lt;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%26lt;0.001) and revealed substantial to nearly perfect interobserver agreement (ICARS-ICC: 0.812; SARA-ICC: 0.854; SARASRT-ICC: 0.724). %26lt;br%26gt;InterpretationEarly-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.

• 出版日期2014-12