An Investigation of the relationship between dystonia severity and functional independence and quality of life in children with dystonia: A Preliminary Study

Objectives: Limitations in activities of daily living and deterioration in quality of life may be observed in individuals with dystonia due to involuntary muscle contractions. The aim of our study was to examine the relationship between dystonia severity and functional independence and quality of life in children with dystonia. Methods: Six children with dystonia aged 8-14 years were included in the study. Individuals' dystonia severity was assessed with the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), functional independence by WeeFIM, and quality of life with the CPCHILD. The relationship between dystonia severity and functional independence and quality of life was analyzed by Spearman correlation analysis. Results: There was a very strong negative correlation between BFMDRS-movement section and the WeeFIM total score, the mobility and transfer subsections, and the total value of CPCHILD, personal care, positioning-transfer-mobility and overall quality of life subsections. There was a very strong negative correlation between the BFMDRS-disability section and WeeFIM total score and self-care, mobility and transfer subsections; the total value of CPCHILD, personal care, positioning-transfer-mobility, and comfort-emotions subscales(p<0.05). Conclusion: As a result of this preliminary study, it was determined that the severity of dystonia in children with dystonia negatively affected their independence in daily living activities and their quality of life. Interventions to reduce the severity of dystonia in children can improve their level of independence and quality of life. In the continuation of the study, there is a need to analyze the results of more patient evaluations and to examine other factors affecting quality of life.
Keywords: Dystonia, children, functional independence, quality of life