title: Correlates of health related quality of life in adult patients with spinal muscular atrophy.
abstract: To improve care for patients with spinal muscular atrophy (SMA), we assessed the physical and mental quality of life (QoL) in 62 adult patients with SMA.
  
  
  
  Physical component scores (PCS) and mental component scores (MCS) of the Short Form-36 Health Survey (SF-36) were obtained. Correlations with demographics, disease severity, and emotional distress were assessed. We used hierarchical multiple regression analysis to identify determinants of QoL.
  
  
  
  PCS scores were lower, and MCS scores higher than in the healthy reference population. Patients with milder SMA types reported lower scores on several MCS domains. Motor skills scores and emotional distress explained 16% of the variance in PCS. SMA type and emotional distress explained 10% and 45% of the variance of MCS, respectively.
  
  
  
  Patients with milder forms of SMA tend to have a reduced mental QoL. Psychological interventions to reduce emotional distress may improve both mental and physical QoL. Muscle Nerve 54: 850-855, 2016.
authors.affiliation: Departments of Rehabilitation, Nursing Science and Sports, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Postbus 85500, 3508 GA, Utrecht, The Netherlands. e.t.kruitwagen@umcutrecht.nl.
authors.forename: Esther Th
authors.initials: ET
authors.lastname: Kruitwagen-Van Reenen
authors.affiliation: Department of Neurology and Neurosurgery, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors.forename: Renske I
authors.initials: RI
authors.lastname: Wadman
authors.affiliation: Departments of Rehabilitation, Nursing Science and Sports, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Postbus 85500, 3508 GA, Utrecht, The Netherlands.
authors.forename: Johanna Ma
authors.initials: JM
authors.lastname: Visser-Meily
authors.affiliation: Department of Neurology and Neurosurgery, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors.forename: Leonard H
authors.initials: LH
authors.lastname: van den Berg
authors.affiliation: Departments of Rehabilitation, Nursing Science and Sports, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Postbus 85500, 3508 GA, Utrecht, The Netherlands.
authors.forename: Carin
authors.initials: C
authors.lastname: Schröder
authors.affiliation: Departments of Rehabilitation, Nursing Science and Sports, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Postbus 85500, 3508 GA, Utrecht, The Netherlands.
authors.forename: W Ludo
authors.initials: WL
authors.lastname: van der Pol
mesh_terms: D000328:Adult; D005260:Female; D006304:Health Status; D006801:Humans; D008297:Male; D008875:Middle Aged; D009134:Muscular Atrophy, Spinal / Q000523:psychology*; D011788:Quality of Life / Q000523:psychology*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires
keywords: SF-36; SMA; adults; neuromuscular diseases; perceived health; quality of life