Motor and non-motor determinants of health-related quality of life in young dystonia patients

• Published in: Parkinsonism & related disorders Vol. 58; pp. 50 - 55
• Main Authors: Eggink, Hendriekje, Coenen, Maraike A., de Jong, Ronald, Toonen, Rivka F., Eissens, Melanie H., Veenstra, Wencke S., Peall, Kathryn J., Sival, Deborah A., Elema, Agnes, Tijssen, Marina AJ
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.01.2019
• Subjects: Adolescent; Adult; Behavioral Symptoms - etiology; Behavioral Symptoms - physiopathology; Child; Cross-Sectional Studies; Dyskinesias - etiology; Dyskinesias - physiopathology; Dystonia; Dystonia - complications; Dystonia - physiopathology; Dystonic Disorders - complications; Dystonic Disorders - physiopathology; Female; Humans; Motor symptoms; Non-motor symptoms; Prospective Studies; Psychiatric; Quality of Life; Severity of Illness Index; Young Adult; Non-motor symptoms; Dystonia; Quality of life; Motor symptoms; Psychiatric
• ISSN: 1353-8020; 1873-5126
• DOI: 10.1016/j.parkreldis.2018.08.008

To systematically investigate the relationship between motor and non-motor symptoms, and health-related quality of life (HR-QoL) in children and young adults with dystonia. In this prospective observational cross-sectional study, 60 patients (6–25 years) with childhood-onset dystonia underwent a multidisciplinary assessment of dystonia severity (Burke-Fahn-Marsden Dystonia Rating Scale, Global Clinical Impression), motor function (Gross Motor Function Measure, Melbourne Assessment of Unilateral Upper Limb Function), pain (visual analogue scale), intelligence (Wechsler Intelligence Scale), executive functioning (Behavior Rating Inventory of Executive Function) and anxiety/depression (Child/Adult Behavior Checklist). Measures were analyzed using a principal component analysis and subsequent multiple regression to evaluate which components were associated with HR-QoL (Pediatric Quality of life Inventory) for total group, and non-lesional (primary) and lesional (secondary) subgroups. Patients (29 non-lesional, 31 lesional dystonia) had a mean age of 13.6 ± 5.9 years. The principal component analysis revealed three components: 1) motor symptoms; 2) psychiatric and behavioral symptoms; and 3) pain. HR-QoL was associated with motor symptoms and psychiatric and behavioral symptoms (R2 = 0.66) for the total sample and lesional dystonia, but in the non-lesional dystonia subgroup only with psychiatric and behavioral symptoms (R2 = 0.51). Non-motor symptoms are important for HR-QoL in childhood-onset dystonia. We suggest a multidisciplinary assessment of motor and non-motor symptoms to optimize individual patient management. •Health-related quality of life is impaired in children and young-adults with dystonia.•Non-motor symptoms are important contributors of quality of life.•Systematic motor and non-motor assessment is necessary to measure disease burden.