Quality of life in patients with myotonic dystrophy type 2

Abstract

© 2016 Elsevier B.V. Aim To analyze quality of life (QoL) in a large cohort of myotonic dystrophy type 2 (DM2) patients in comparison to DM1 control group using both generic and disease specific questionnaires. In addition, we intended to identify different factors that might affect QoL of DM2 subjects. Patients and method 49 DM2 patients were compared with 42 adult-onset DM1 patients. Patients completed SF-36 questionnaire and individualized neuromuscular quality of life questionnaire (INQoL). Following measures were also included: Medical Research Council 0-5 point scale for muscle strength, Addenbrooke's cognitive examination revised for cognitive status, Hamilton rating scale for depression, Krupp's fatigue severity scale and daytime sleepiness scale (DSS) Results SF-36 total score and physical composite score did not differ between DM1 and DM2 patients (p > 0.05). However, role emotional and mental composite score were better in DM2 (p < 0.05). INQoL total score was similar in both groups (p > 0.05), although DM2 patients showed less impairment in independence (p < 0.05) and body image domains (p < 0.01). Regarding symptoms assessed by INQoL, DM2 patients showed less severe complaint of myotonia (p < 0.01). Multiple linear regression analysis showed that significant predictors of worse QoL in DM2 patients were older age, worse muscle strength and higher level of fatigue. Conclusion QoL reports of DM2 patients with the most severe form of the disease are comparable to those of DM1 patients. Special attention of clinicians should be paid to DM2 patients with older age, more severe muscle weakness and higher level of fatigue since they may be at higher risk to have worse QoL.