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

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 ±â€¯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.

[Outcomes after hand injuries]. / Uitkomsten van ernstige handletsels.

A 25-year-old male and a 41-year-old male presented at the Emergency Department with a hand injury. Although both patients received immediate surgical care and specialized rehabilitation care, outcomes were better in the first patient, who had sustained the more severe hand injury. Outcomes were influenced not only by injury severity, but also by personal and external factors. Personal factors, such as age, motivation and compliance, were more favourable in the first patient. External factors, such as complications, delayed care, language problems and social problems, mainly affected the second patient, who was less severely injured. As a result, outcomes on all functional levels of the WHO's International Classification of Functioning, Disability and Health (body functions and structure, and activity and participation) were better in the patient with the more severe injury. Specialized hand care is required in hand injuries, but professionals should also be aware that personal and external factors have a substantial influence on outcomes after hand injuries.