title: Significant impact of behavioral and cognitive impairment on quality of life in patients with myotonic dystrophy type 1.
abstract: OBJECTIVE
  To assess an impact of cognitive and behavioral impairment on QoL in a larger cohort of patients with DM1.
  
  
  METHODS
  Sixty six genetically confirmed DM1 patients (22 with juvenile (jDM1) and 44 with adult form (aDM1) of the disease) were recruited. Following behavioral tests were used: Hamilton scales for depression and anxiety (HamD and HamA), Daytime Sleepiness Scale (DSS), and Krupp's Fatigue Severity Scale (FSS). Patients also underwent detailed classic neuropsychological investigation and Cambridge Neuropsychological Test Automated Battery (CANTAB). Individualized Neuromuscular Quality of Life questionnaire (INQoL) was used as a measure of QoL.
  
  
  RESULTS
  Patients with jDM1 scored lower than aDM1 patients regarding total INQoL score and all INQoL subdomains, except for myotonia. Significant predictors of total INQoL score in patients with jDM1 were severity of fatigue (β=+0.60, p<0.01) and percentage of correct responses on Spatial Recognition Memory test from CANTAB that measures visuospatial abilities (β=-0.38, p<0.05). The most important predictors of total INQoL score in patients with aDM1 were severity of fatigue (β=+0.36, p<0.05) and level of education (β=-0.29, p<0.05).
  
  
  CONCLUSIONS
  Our results showed clear influence of different central manifestations on QoL in patients with both aDM1 and jDM1.
authors.affiliation: Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
authors.forename: V
authors.initials: V
authors.lastname: Rakocevic-Stojanovic
authors.affiliation: Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia. Electronic address: stojanperic@gmail.com.
authors.forename: S
authors.initials: S
authors.lastname: Peric
authors.affiliation: Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
authors.forename: R
authors.initials: R
authors.lastname: Madzarevic
authors.affiliation: Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
authors.forename: V
authors.initials: V
authors.lastname: Dobricic
authors.affiliation: Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
authors.forename: V
authors.initials: V
authors.lastname: Ralic
authors.affiliation: Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
authors.forename: V
authors.initials: V
authors.lastname: Ilic
authors.affiliation: Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
authors.forename: I
authors.initials: I
authors.lastname: Basta
authors.affiliation: Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
authors.forename: A
authors.initials: A
authors.lastname: Nikolic
authors.affiliation: Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
authors.forename: E
authors.initials: E
authors.lastname: Stefanova
mesh_terms: D000328:Adult; D017668:Age of Onset; D003072:Cognition Disorders / Q000209:etiology / Q000503:physiopathology*; D015331:Cohort Studies; D004522:Educational Status; D005221:Fatigue / Q000209:etiology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications / Q000503:physiopathology*; D011788:Quality of Life; D055815:Young Adult
keywords: Behavior; Cognition; Fatigue; Myotonic dystrophy type 1; Quality of life