PMID- 27696366 OWN - NLM STAT- MEDLINE DCOM- 20170117 LR - 20220331 IS - 1600-0404 (Electronic) IS - 0001-6314 (Linking) VI - 134 IP - 5 DP - 2016 Nov TI - Five-year study of quality of life in myotonic dystrophy. PG - 346-351 LID - 10.1111/ane.12549 [doi] AB - Background - Myotonic dystrophy type 1 (DM1) is the most common muscular dystrophy in adults. There is a complete lack of studies that assessed quality of life (QoL) trajectory during time in DM1 cohorts. Aim - To analyze changes of QoL in patients with DM1 during a 5-year follow-up period and to assess responsiveness of the SF-36 questionnaire. Patients and Method - At the baseline, this study comprised 84 DM1 patients, of whom 62 were retested after the mean period of 64.2 +/- 3.9 months. Severity of muscular weakness was assessed using the Muscular Impairment Rating Scale (MIRS). Patients completed Serbian version of the SF-36 questionnaire as a measure of health-related QoL. Results - After 5 years, MIRS score of our DM1 patients showed significant progression of 0.5 grade (P < 0.01). All mental subdomains, role physical, and total SF-36 scores significantly improved after 5 years (P < 0.01). Unexpectedly, worsening of muscular weakness from mild to severe was in association with improvement of QoL. Conclusion - QoL improved in our cohort of DM1 patients during a 5-year period despite the progression of the disease. SF-36 should be used with caution as a patient-reported outcome measure in DM1 clinical trials. CI - (c) 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. FAU - Peric, S AU - Peric S AD - Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. stojanperic@gmail.com. FAU - Vujnic, M AU - Vujnic M AD - Department of Pathophysiology, Faculty of Medicine, University of Banja Luka, Banja Luka, Republic of Srpska, Bosnia and Herzegovina. FAU - Dobricic, V AU - Dobricic V AD - Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. FAU - Marjanovic, A AU - Marjanovic A AD - Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. FAU - Basta, I AU - Basta I AD - Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. FAU - Novakovic, I AU - Novakovic I AD - Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. AD - Institute of Human Genetics, School of Medicine, University of Belgrade, Belgrade, Serbia. FAU - Lavrnic, D AU - Lavrnic D AD - Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. FAU - Rakocevic-Stojanovic, V AU - Rakocevic-Stojanovic V AD - Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. LA - eng PT - Journal Article DEP - 20151221 PL - Denmark TA - Acta Neurol Scand JT - Acta neurologica Scandinavica JID - 0370336 SB - IM MH - Adult MH - *Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Myotonic Dystrophy/*physiopathology MH - *Quality of Life OTO - NOTNLM OT - SF-36 OT - follow-up OT - myotonic dystrophy type 1 OT - quality of life OT - responsiveness EDAT- 2016/10/04 06:00 MHDA- 2017/01/18 06:00 CRDT- 2016/10/04 06:00 PHST- 2015/11/24 00:00 [accepted] PHST- 2016/10/04 06:00 [entrez] PHST- 2016/10/04 06:00 [pubmed] PHST- 2017/01/18 06:00 [medline] AID - 10.1111/ane.12549 [doi] PST - ppublish SO - Acta Neurol Scand. 2016 Nov;134(5):346-351. doi: 10.1111/ane.12549. Epub 2015 Dec 21.