Significant impact of behavioral and cognitive impairment on quality of life in patients with myotonic dystrophy type 1

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 (beta = +0.60, p < 0.01) and percentage of correct responses on Spatial Recognition Memory test from CANTAB that measures visuospatial abilities (beta = -0.38, p < 0.05). The most important predictors of total INQoL score in patients with aDM1 were severity of fatigue (beta = +0.36, p < 0.05) and level of education (beta = -0.29, p < 0.05). Conclusion: Our results showed clear influence of different central manifestations on QoL in patients with both aDM1 and jDM1. (C) 2014 Elsevier B.V. All rights reserved.