title: Quality of life in patients with myotonic dystrophy type 2.
abstract: OBJECTIVE
  To analyze quality of life (QoL) in a large cohort of myotonic dystrophy type 2 (DM2) patients in comparison to DM1 control group using both generic and disease specific questionnaires. In addition, we intended to identify different factors that might affect QoL of DM2 subjects.
  
  
  METHODS
  49 DM2 patients were compared with 42 adult-onset DM1 patients. Patients completed SF-36 questionnaire and individualized neuromuscular quality of life questionnaire (INQoL). Following measures were also included: Medical Research Council 0-5 point scale for muscle strength, Addenbrooke's cognitive examination revised for cognitive status, Hamilton rating scale for depression, Krupp's fatigue severity scale and daytime sleepiness scale (DSS) RESULTS: SF-36 total score and physical composite score did not differ between DM1 and DM2 patients (p>0.05). However, role emotional and mental composite score were better in DM2 (p<0.05). INQoL total score was similar in both groups (p>0.05), although DM2 patients showed less impairment in independence (p<0.05) and body image domains (p<0.01). Regarding symptoms assessed by INQoL, DM2 patients showed less severe complaint of myotonia (p<0.01). Multiple linear regression analysis showed that significant predictors of worse QoL in DM2 patients were older age, worse muscle strength and higher level of fatigue.
  
  
  CONCLUSIONS
  QoL reports of DM2 patients with the most severe form of the disease are comparable to those of DM1 patients. Special attention of clinicians should be paid to DM2 patients with older age, more severe muscle weakness and higher level of fatigue since they may be at higher risk to have worse QoL.
authors.affiliation: Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. Electronic address: stojan.peric@gmail.com.
authors.forename: Vidosava
authors.initials: V
authors.lastname: Rakocevic Stojanovic
authors.affiliation: Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia.
authors.forename: Stojan
authors.initials: S
authors.lastname: Peric
authors.affiliation: General Hospital "Djordje Joanovic", Zrenjanin, Serbia.
authors.forename: Teodora
authors.initials: T
authors.lastname: Paunic
authors.affiliation: Center for Human Genetics, Faculty of Biology, University of Belgrade, Belgrade, Serbia.
authors.forename: Jovan
authors.initials: J
authors.lastname: Pesovic
authors.affiliation: Department of Pathophysiology, Faculty of Medicine, University of Banja Luka, Banja Luka, Republic of Srpska, Bosnia and Herzegovina.
authors.forename: Milorad
authors.initials: M
authors.lastname: Vujnic
authors.affiliation: Institute for Health Protection of Mother and Child of Serbia "Dr. Vukan Cupic", Belgrade, Serbia.
authors.forename: Marina
authors.initials: M
authors.lastname: Peric
authors.affiliation: Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia.
authors.forename: Ana
authors.initials: A
authors.lastname: Nikolic
authors.affiliation: Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia.
authors.forename: Dragana
authors.initials: D
authors.lastname: Lavrnic
authors.affiliation: Center for Human Genetics, Faculty of Biology, University of Belgrade, Belgrade, Serbia.
authors.forename: Dusanka
authors.initials: D
authors.lastname: Savic Pavicevic
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000175:diagnosis / Q000523:psychology*; D011788:Quality of Life / Q000523:psychology*; D011795:Surveys and Questionnaires
keywords: Fatigue; INQoL; Myotonic dystrophy type 2; Quality of life; SF-36