Spits, A.H. (2012) Validatie van ataxieschalen bij gezonde kinderen. thesis, Medicine.
Full text available on request.Abstract
Background: Early onset ataxia (EOA) refers to heterogeneous degenerative and metabolic diseases, often of autosomal-recessive inheritance. To obtain insight in epidemiology, phenotypic occurrence, disease progression and, eventually, potential treatment, a longitudinal European EOA database will be set up, covering both pediatric and adult disease progression. Ataxia can be assessed by ataxia rating scales (involving ICARS, SARA and BARS). However, so far, these scales have not adequately been validated in children. In the present study, we therefore aimed to assess the association between age and ataxia rating scales in healthy children. Furthermore, we aimed to determine the inter-observer, intra-observer and test-retest reliability for ICARS, SARA and BARS. Methods: We included 52 healthy children between 4 and 16 years, with two boys and two girls per year of age. From each child, we collected information on age, gender, sport activities, school achievement and education level of the parents. We videotaped all 52 children during the performance of a combined ataxia rating scale test (PARS, consisting of ICARS, SARA, BARS and a nine-hole pegtest). Twelve children were videotaped twice. Three observers of the department of pediatric neurology, independently assessed all video recordings. From these data, we obtained: age- and gender dependency, interobserver, intra-observer and test-retest reliability for each of the three rating-scales. We also determined the correlation between ICARS, SARA, BARS and the nine-hole peg test. Results: All three ataxia rating scales revealed a significant age dependent effect up to the age of ten. After correcting the scales for the age dependent effect, a significant gender dependent effect was found for ICARS and BARS. The inter-observer reliability was high, with intra-class correlation coefficients (ICC’s) of 0.85, 0.72 and 0.69 for ICARS; SARA and BARS, respectively. Bland-Altman plots revealed most consistent outcomes when ICARS and SARA rating scales were applied in children older than six years. The ICC’s for intra-observer reliability were 0.86-0.94, 0.92-098 and 0.70-083 for ICARS, SARA and BARS, respectively. The Bland-Altman plots correspond to the ICC’s. The ICC’s for test-retest reliability were 0.98, 0.62 and 0.92 for ICARS; SARA and BARS, respectively. For test-retest reliability, Bland-Altman plots reveal minor differences between de scales. Correlating ataxia scales and the nine-hole peg test to each other, revealed Spearman rank correlation coefficients between 0.65 and 0.88. Conclusion: In healthy children under the age of ten years, ataxia rating scales are age and (partially) gender dependent. Comparing ataxia rating scales revealed that ICARS, SARA, BARS and the nine-hole peg tests are related. However, BARS showed the least reliable results in children. In healthy children, present data implicate that both ICARS and SARA are reliable tests, especially when applied in children of six years and older.
| Item Type: | Thesis (Thesis) |
|---|---|
| Supervisor name: | Sival, dr. D. and kinderneurologie, UMCG |
| Faculty: | Medical Sciences |
| Date Deposited: | 25 Jun 2020 10:41 |
| Last Modified: | 25 Jun 2020 10:41 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/303 |
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