Hendriekje Eggink1, Maraike A Coenen1, Ronald de Jong2, Rivka F Toonen2, Melanie H Eissens1, Wencke S Veenstra1, Kathryn J Peall3, Deborah A Sival1, Agnes Elema2, Marina Aj Tijssen4. 1. University of Groningen, University Medical Center Groningen, Department of Neurology, Groningen, The Netherlands. 2. University of Groningen, University Medical Center Groningen, Department of Rehabilitation Medicine, Center for Rehabilitation, Groningen, The Netherlands. 3. University of Groningen, University Medical Center Groningen, Department of Neurology, Groningen, The Netherlands; Institute of Psychological Medicine and Clinical Neurosciences, Hadyn Ellis Building, Cardiff University, Cardiff, United Kingdom. 4. University of Groningen, University Medical Center Groningen, Department of Neurology, Groningen, The Netherlands. Electronic address: m.a.j.de.koning-tijssen@umcg.nl.
Abstract
OBJECTIVES: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.
OBJECTIVES: 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. METHODS: 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. RESULTS: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). CONCLUSIONS: 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.
Authors: Damiana Scuteri; Laura Rombolà; Silvia Natoli; Antonio Pisani; Paola Bonsi; Chizuko Watanabe; Giacinto Bagetta; Paolo Tonin; Maria Tiziana Corasaniti Journal: Life (Basel) Date: 2021-01-12
Authors: Francisco Pereira da Silva-Júnior; Camila Oliveira Dos Santos Alves; Sônia Maria Cesar Azevedo Silva; Vanderci Borges; Henrique Ballalai Ferraz; Maria Sheila Guimarães Rocha; João Carlos Papaterra Limongi; Egberto Reis Barbosa; Patrícia de Carvalho Aguiar Journal: Neurol Sci Date: 2021-07-23 Impact factor: 3.307