Assessment of speech in early-onset ataxia: a pilot study
Article first published online: 18 JUN 2014
© 2014 Mac Keith Press
Developmental Medicine & Child Neurology
Volume 56, Issue 12, pages 1202–1206, December 2014
How to Cite
Kuiper, M. J., Brandsma, R., Lawerman, T. F., Lunsing, R. J., Keegstra, A. L., Burger, H., De Koning, T. J., Tijssen, M. A. J. and Sival, D. A. (2014), Assessment of speech in early-onset ataxia: a pilot study. Developmental Medicine & Child Neurology, 56: 1202–1206. doi: 10.1111/dmcn.12517
- Issue published online: 12 NOV 2014
- Article first published online: 18 JUN 2014
- Manuscript Accepted: 24 APR 2014
- European Paediatric Neurology Society
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).
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.
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).
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.