Lawerman Tjitske F, Brandsma Rick, Burger Huibert, Burgerhof Johannes G M, Sival Deborah A
Department of Neurology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Department of General Medicine, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Dev Med Child Neurol. 2017 Oct;59(10):1077-1082. doi: 10.1111/dmcn.13507. Epub 2017 Aug 17.
For reliable assessment of ataxia severity in children, the Childhood Ataxia and Cerebellar Group of the European Pediatric Neurology Society aimed to validate the Scale for Assessment and Rating of Ataxia (SARA) according to age.
Twenty-two pediatric ataxia experts from 15 international institutions scored videotaped SARA performances in 156 typically developing children (4-16y: m/f=1; 12 children per year of age; including nine different nationalities). We determined age-dependency and reliability of pediatric SARA scores by a mixed model.
In typically developing children, age was the only variable that revealed a relationship with SARA scores (p<0.001). The youngest children revealed the highest scores and the highest variation in scores (<8y; p<0.001). After 11 years of age, pediatric scores approached adult outcomes. The interobserver agreement of total SARA scores was substantial with an intraclass correlation coefficient of 0.63 (95% confidence interval 0.56-0.69; p<0.001).
In typically developing European children, both SARA scores and interobserver agreement are age-dependent. For reliable interpretation of pediatric SARA scores, consideration of the underlying test construct appears prudent. These data will hopefully contribute to a correct and uniform interpretation of longitudinal SARA scores from childhood to adulthood.
为了可靠地评估儿童共济失调的严重程度，欧洲儿科神经病学学会儿童共济失调与小脑疾病研究组旨在根据年龄对共济失调评估与评分量表（SARA）进行验证。
来自15个国际机构的22名儿科共济失调专家对156名发育正常儿童（4至16岁：男/女=1；每个年龄段12名儿童；包括9个不同国籍）的SARA表现录像进行评分。我们通过混合模型确定儿科SARA评分的年龄依赖性和可靠性。
在发育正常的儿童中，年龄是唯一与SARA评分相关的变量（p<0.001）。年龄最小的儿童得分最高且分数变化最大（<8岁；p<0.001）。11岁以后，儿科评分接近成人结果。SARA总分的观察者间一致性较高，组内相关系数为0.63（95%置信区间0.56 - 0.69；p<0.001）。
在发育正常的欧洲儿童中，SARA评分和观察者间一致性均与年龄有关。为了可靠地解读儿科SARA评分，考虑潜在的测试结构似乎是谨慎的做法。这些数据有望有助于对从儿童期到成年期的纵向SARA评分进行正确和统一的解读。
