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European pediatric normative values for the scale for assessment and rating of ataxia (SARA)

T.F. Lawerman, R. Brandsma, R.J. Lunsing, J.G.M. Burgerhof, D.A. Sival, On behalf of the Childhood Ataxia and Cerebellar Study Group of the European Pediatric Neurology Society, N. Barisic, P. Baxter, E. Bertini, L. Blumkin, V. Brankovic, G.E. Calabro, C.E. Catsman-Berrevoets, D. Craiu, B. Dan, A. Dica, T. Franciskovic, J. Gburek-Augustat, S. Grunt, H. Hartley, F. Kammoun, C. Kennedy, M.J. Kuiper, I. Lehman, A. Lustenberger, F. Mancini, M. Mirabelli-Badenier, E. Mulder-den Hartog, M. Steinlin, M. Synofzik, C.C. Triki, E.M. Valente, G. Vasco, A. Zekavica (Groningen, Netherlands)

Meeting: 2016 International Congress

Abstract Number: 1080

Keywords: Ataxia: Treatment, Cerebellum, Development, Early-onset cerebellar ataxia(EOCA)

Session Information

Date: Wednesday, June 22, 2016

Session Title: Ataxia

Session Time: 12:00pm-1:30pm

Location: Exhibit Hall located in Hall B, Level 2

Objective: To obtain international age-related normative values for the SARA in healthy European children.

Background: The Scale for Assessment and Rating of Ataxia (SARA) is designed in adults as a uniform, reliable and ubiquitous biomarker for quantitative assessment of ataxia from pediatric to adult life. Although SARA is reliably applicable in adults, we have shown that pediatric outcomes can also be influenced by the age of the child. For uniform longitudinal interpretation of pediatric SARA scores, normative values should be obtained in healthy children, first. In a large European cohort of healthy children (4-16 years of age), we therefore aimed to determine normative age-related SARA scores.

Methods: Twenty-two pediatric neurologists, from nine countries (CACG-EPNS members) independently scored 156 healthy children from nine different European nationalities (n=12/year; m:f=1; age range 4-16 years). We determined SARA age-dependency and international inter-observer agreement on pediatric SARA scores.

Results: In healthy children, SARA scores revealed age dependency (R²=0.47). The youngest children showed the highest scores and also the most variation in scores (≤ 7 years; p<0.001). After 12 years of age, children approached adult optimum SARA values. The inter-observer agreement was substantial (Intraclass Correlation Coefficient: 0.69), revealing a positive relationship with age (p<0.01).

Conclusions: Our data in healthy European children indicate that SARA scores and inter-observer agreement are age dependent. For longitudinal pediatric trials, these findings may implicate that SARA outcomes and cut off points for treatment effects should be interpreted for the age of the child. We hope that forthcoming insight in the pediatric SARA construct will contribute to adequate interpretation of SARA scores from pediatric to adult life.

The results of the completed trial have never been presented, thus far. Pre-liminary results of the incompleted trial have previously been presented at: 10th European Pediatric Neurology Society Congress, Brussels, Belgium, September 2013; Inspiring Infancy, Groningen, Netherlands, May 2014; 4th International Symposium on Pediatric Movement Disorders, Barcelona Spain, February 2015; 19th International Congress of Parkinson’s disease and Movement Disorders, San Diego, USA, June 2015.

To cite this abstract in AMA style:

T.F. Lawerman, R. Brandsma, R.J. Lunsing, J.G.M. Burgerhof, D.A. Sival, On behalf of the Childhood Ataxia and Cerebellar Study Group of the European Pediatric Neurology Society, N. Barisic, P. Baxter, E. Bertini, L. Blumkin, V. Brankovic, G.E. Calabro, C.E. Catsman-Berrevoets, D. Craiu, B. Dan, A. Dica, T. Franciskovic, J. Gburek-Augustat, S. Grunt, H. Hartley, F. Kammoun, C. Kennedy, M.J. Kuiper, I. Lehman, A. Lustenberger, F. Mancini, M. Mirabelli-Badenier, E. Mulder-den Hartog, M. Steinlin, M. Synofzik, C.C. Triki, E.M. Valente, G. Vasco, A. Zekavica. European pediatric normative values for the scale for assessment and rating of ataxia (SARA) [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/european-pediatric-normative-values-for-the-scale-for-assessment-and-rating-of-ataxia-sara/. Accessed May 18, 2025.
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