Influence of multisystemic affection on health-related quality of life in patients with myotonic dystrophy type 1.
OBJECTIVE
To assess health-related quality of life (HRQoL) in patients with DM1, to identify muscular, multisystemic, central and social factors that may affect QoL and to define a DM1 patient in risk of poor QoL.


METHODS
This cross-sectional study comprised 120 DM1 consecutive patients. The following scales were used: Multidimensional Scale of Perceived Social Support (MSPSS), Muscular Impairment Rating Scale (MIRS), battery of neuropsychological tests, acceptance of illness scale (AIS), Hamilton rating scale for depression (Ham-D), Krupp's Fatigue Severity Scale (FSS), Daytime Sleepiness Scale (DSS) and SF-36 questionnaire.


RESULTS
HRQoL was impaired in DM1 patients in both physical and mental domains (PCS was 41.8±23.5, MCS 47.0±24.3 and total SF-36 score 45.6±24.0). The most significant factors correlating with better SF-36 total score were younger age (β=-0.45, p<0.001), shorter duration of disease (β=-0.27, p=0.001), higher education (β=0.20, p=0.009), less severe muscular weakness (β=-0.52, p<0.001), normal swallowing (β=0.22, p=0.005), absence of fainting (β=0.31, p=0.002), absence of snoring (β=0.21, p=0.036), better acceptance of disease (β=-0.17, p=0.036), lower depressiveness (β=-0.46, p=0.001), lower fatigue (β=-0.32, p=0.001), absence of cataract (β=-0.21, p=0.034), absence of kyphosis (β=0.31, p=0.004) and absence of constipation (β=0.24, p=0.016). Second linear regression analysis revealed that depressed (β=-0.38, p<0.001) and elder patients (β=-0.27, p=0.007) and as well as those with poor acceptance of illness (β=-0.21, p=0.006) were in especially higher risk of having poor HRQoL (R(2)=0.68).


CONCLUSIONS
We identified different central, social, muscular, cardiorespiratory and other factors correlating with HRQoL. It is of great importance that most of these factors are amenable to treatment.
Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. stojanperic@gmail.com
S
S
Peric
V Rakocevic
VR
Stojanovic
I
I
Basta
M
M
Peric
M
M
Milicev
S
S
Pavlovic
D
D
Lavrnic
D000328:Adult; D002490:Central Nervous System / Q000503:physiopathology; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology; D003430:Cross-Sectional Studies; D003863:Depression / Q000150:complications / Q000523:psychology; D004522:Educational Status; D004576:Electromyography; D005221:Fatigue / Q000150:complications / Q000523:psychology; D005260:Female; D006321:Heart / Q000503:physiopathology; D006801:Humans; D016014:Linear Models; D008297:Male; D017533:Marital Status; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000503:physiopathology; D009223:Myotonic Dystrophy / Q000503:physiopathology* / Q000523:psychology*; D009483:Neuropsychological Tests; D009790:Occupations; D011788:Quality of Life; D012137:Respiratory System / Q000503:physiopathology; D012306:Risk; D012959:Socioeconomic Factors; D011795:Surveys and Questionnaires