De toepasbaarheid van ataxieschalen tijdens de motorische ontwikkeling van gezonde kinderen

Kuiper, M.J. (2013) De toepasbaarheid van ataxieschalen tijdens de motorische ontwikkeling van gezonde kinderen. thesis, Medicine.

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Abstract

Introduction: When ataxia manifests before the age of 25 it is called Early Onset Ataxia (EOA). To acquire insight in phenotypic spectrum, disease progression and potential treatment options of this relative rare heterogeneous group of diseases, a longitudinal European EOA database is desirable. Ataxia can be quantified by ataxia rating scales (ICARS, SARA and BARS). A pilot study in healthy children assessed the effect of age on the rating scales. ICARS, SARA, BARS and a 9-hole PEG board test were, in this pilot study, combined into a new scale called cPARS. In healthy children, all three ataxia rating scales revealed an age dependent effect. Although, the interobserver reliability in total ataxia scores was high, it was low for the subscore speech. In this present study, we have three aims. First we aimed to determine whether the results of the separately taken ataxia scores (ICARS and SARA), differ from the results of ataxia scores extracted from the combined scale. The results from the previous pilot study, mentioned above, were interpreted under this premise. Secondly, we questioned if the development of the individual subscores (gait, kinetic function, speech and oculomotor function) reflect the maturation of the central nervous system. Thirdly, we questioned whether the poor interobserver agreement of speech was a consequence of scoring skills of the observers or a consequence of the moderate ability to score as a result of the ataxia guidelines. Methods: Part 1: ICARS, SARA and cPARS were video-recorded, in a randomized sequence, in 13 healthy children, aged 4-16 years (1 child per year of age). The video-fragments were assessed by three observers from the department of (pediatric) neurology. We compared the results of the separately taken ataxia scales with the results of the combined ataxia scale. Part 2: Scores of cPARS from 52 healthy children, from existing data, were used to determine the association between the subscores and age. Then we determined in which sequence the subscores develop. Part 3: The subscore speech of cPARS in 52 healthy children is analyzed twice, first by 3 (pediatric) neurologists and a speech therapist with video footage of the child. Secondly by 3 (pediatric) neurologists based on sound alone (exclusion of video footage). We determined the interobserver agreement and compared the two methods and the observers. Results: Part 1: Separately taken ICARS and SARA showed no significant difference with ICARS and SARA extracted from the combined cPARS (p>.05). Part 2: The subscores speech, gait and kinetic function were age-dependent and reached the optimum score in chronological sequence (at 8.5, 9 and 12 years, resp.) Part 3: The Intraclass Correlation Coefficients (ICCs) for interobserver reliability were.403, .222 and .512 in the first scores (fair to moderate in ICARS, SARA and BARS respectively) and 400, .337 and .357 in the second scores (fair in ICARS, SARA and BARS respectively). There was an effect of method (p<.05), but no effect of observer. Conclusion: Part 1: cPARS is a representative test, from which individual ataxia rating scales can be extracted. This implies that the premise, mentioned above, is correct, concluding that ataxia rating scales in healthy children are age-dependant. Part 2: The chronologic sequence in which the subscore develop reflects the maturation of the central nervous system. Part 3: The poor interobserver reliability for the subscore speech is a consequence of the moderate ability to score as a result of the ataxia guidelines. In young children, longitudinal interpretation of ataxia parameters should occur with age-validated ataxia rating scales.

Item Type:	Thesis (Thesis)
Supervisor name:	Sival, Dr. D.A.
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 10:40
Last Modified:	25 Jun 2020 10:40
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/159

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