Applicability of quantitative oculomotor and SARA assessment in children

Abstract

Background: In clinical practice, eye movements can provide an early diagnostic marker for early onset ataxia (EOA). However, quantitative oculomotor assessment is not included in the most frequently used and age-validated ataxia rating scale in children, the Scale for the Assessment and Rating of Ataxia (SARA). We aimed to investigate the applicability of semi-quantitative eye movement assessment by the International Cooperative Ataxia Rating Scale (ICARS_OCM) and Ocular Motion Score (OMS_7-10) complementary to SARA measurements in children.

Methods: In 52 typically developing children (aged 4-16 years; n = 4 per year of age), three independent assessors scored saccadic eye movements and ocular pursuit according to the ICARS_OCM and matching parameters from the OMS_7-10. For ICARS_OCM, we determined 1) construct validity for coordinated eye movements by correlation with OMS_7-10, ICARS_{EYE-HAND-COORDINATION} and SARA subscale scores, 2) agreement percentage and inter-rater agreement (Fleiss Kappa) and 3) age-dependency.

Results: Spearman's rank correlations of ICARS_OCM with OMS_7-10 and ICARS- and SARA subscales were moderate to fair (all p < .001). Inter-rater agreement of ICARS-_OCM was 80.8%; (Fleiss Kappa: 0.411). ICARS_OCM scores revealed a similar exponentially decreasing association with age as the other SARA (sub)scores, reaching a plateau at 10 years of age.

Interpretation: ICARS_OCM has a valid construct for the measurement of coordinated eye movement performance and is reliably assessable in children. ICARS_OCM reveals a similar age-dependent relationship as the other ataxia subscales, reflecting the physiological maturation of the cerebellum. In children, these data may implicate that ICARS_OCM can reliably contribute to coordination assessment, complementary to the SARA subscales.

Keywords: Ataxia rating scale; Cerebellum; Child; ICARS; Oculomotor assessment; SARA.