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

V. Rakocevic-Stojanovic; S. Peric; R. Madzarevic; V. Dobricic; V. Ralic; V. Ilic; I. Basta; A. Nikolic; E. Stefanova

doi:10.1016/j.clineuro.2014.08.021

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

V. Rakocevic-Stojanovic, S. Peric, R. Madzarevic, V. Dobricic, V. Ralic, V. Ilic, I. Basta, A. Nikolic, E. Stefanova

Institute of Neurogenetics

University of Belgrade

33 Citations (Scopus)

Abstract

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.

Objective To assess an impact of cognitive and behavioral impairment on QoL in a larger cohort of patients with DM1.

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).

Conclusion Our results showed clear influence of different central manifestations on QoL in patients with both aDM1 and jDM1.

Original language	English
Journal	Clinical Neurology and Neurosurgery
Volume	126
Pages (from-to)	76-81
Number of pages	6
ISSN	0303-8467
DOIs	https://doi.org/10.1016/j.clineuro.2014.08.021
Publication status	Published - 11.2014

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@article{214cc38cf879419296c688bda5d21341,

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

abstract = "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.Objective To assess an impact of cognitive and behavioral impairment on QoL in a larger cohort of patients with DM1.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).Conclusion Our results showed clear influence of different central manifestations on QoL in patients with both aDM1 and jDM1.",

author = "V. Rakocevic-Stojanovic and S. Peric and R. Madzarevic and V. Dobricic and V. Ralic and V. Ilic and I. Basta and A. Nikolic and E. Stefanova",

year = "2014",

month = nov,

doi = "10.1016/j.clineuro.2014.08.021",

language = "English",

volume = "126",

pages = "76--81",

journal = "Clinical Neurology and Neurosurgery",

issn = "0303-8467",

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TY - JOUR

T1 - Significant impact of behavioral and cognitive impairment on quality of life in patients with myotonic dystrophy type 1

AU - Rakocevic-Stojanovic, V.

AU - Peric, S.

AU - Madzarevic, R.

AU - Dobricic, V.

AU - Ralic, V.

AU - Ilic, V.

AU - Basta, I.

AU - Nikolic, A.

AU - Stefanova, E.

PY - 2014/11

Y1 - 2014/11

N2 - 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.Objective To assess an impact of cognitive and behavioral impairment on QoL in a larger cohort of patients with DM1.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).Conclusion Our results showed clear influence of different central manifestations on QoL in patients with both aDM1 and jDM1.

AB - 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.Objective To assess an impact of cognitive and behavioral impairment on QoL in a larger cohort of patients with DM1.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).Conclusion Our results showed clear influence of different central manifestations on QoL in patients with both aDM1 and jDM1.

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DO - 10.1016/j.clineuro.2014.08.021

M3 - Journal articles

AN - SCOPUS:84907516139

SN - 0303-8467

VL - 126

SP - 76

EP - 81

JO - Clinical Neurology and Neurosurgery

JF - Clinical Neurology and Neurosurgery

ER -

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

Abstract

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