Influence of multisystemic affection on health-related quality of life in patients with myotonic dystrophy type 1

• Published in: Clinical neurology and neurosurgery Vol. 115; no. 3; pp. 270 - 275
• Main Authors: Peric, S, Stojanovic, V. Rakocevic, Basta, I, Peric, M, Milicev, M, Pavlovic, S, Lavrnic, D
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01-03-2013; Elsevier Limited
• Subjects: Acceptance of illness; Adult; Central Nervous System - physiopathology; Cognition Disorders - etiology; Cognition Disorders - psychology; Constipation; Cross-Sectional Studies; Depression; Depression - complications; Depression - psychology; Educational Status; Electromyography; Fatigue; Fatigue - complications; Fatigue - psychology; Female; Health-related quality of life; Heart - physiopathology; Humans; Illnesses; Linear Models; Male; Marital Status; Middle Aged; Muscle, Skeletal - physiopathology; Muscular dystrophy; Myotonic dystrophy; Myotonic Dystrophy - physiopathology; Myotonic Dystrophy - psychology; Myotonic dystrophy type 1; Neurology; Neuropsychological Tests; Neurosurgery; Occupations; Quality of Life; Regression analysis; Respiratory System - physiopathology; Risk; Social support; Socioeconomic Factors; Surveys and Questionnaires; Acceptance of illness; Myotonic dystrophy type 1; Health-related quality of life; Depression
• ISSN: 0303-8467; 1872-6968
• DOI: 10.1016/j.clineuro.2012.05.015

Abstract Aim 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. Patients and method 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 ( R2 = 0.68). Conclusion 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.