Vidosava Rakocevic Stojanovic1, Stojan Peric2, Teodora Paunic3, Jovan Pesovic4, Milorad Vujnic5, Marina Peric6, Ana Nikolic2, Dragana Lavrnic2, Dusanka Savic Pavicevic4. 1. Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. Electronic address: stojan.peric@gmail.com. 2. Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. 3. General Hospital "Djordje Joanovic", Zrenjanin, Serbia. 4. Center for Human Genetics, Faculty of Biology, University of Belgrade, Belgrade, Serbia. 5. Department of Pathophysiology, Faculty of Medicine, University of Banja Luka, Banja Luka, Republic of Srpska, Bosnia and Herzegovina. 6. Institute for Health Protection of Mother and Child of Serbia "Dr. Vukan Cupic", Belgrade, Serbia.
Abstract
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.
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 DM2patients were compared with 42 adult-onset DM1patients. 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 DM2patients (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 DM2patients showed less impairment in independence (p<0.05) and body image domains (p<0.01). Regarding symptoms assessed by INQoL, DM2patients showed less severe complaint of myotonia (p<0.01). Multiple linear regression analysis showed that significant predictors of worse QoL in DM2patients were older age, worse muscle strength and higher level of fatigue. CONCLUSION: QoL reports of DM2patients with the most severe form of the disease are comparable to those of DM1patients. Special attention of clinicians should be paid to DM2patients with older age, more severe muscle weakness and higher level of fatigue since they may be at higher risk to have worse QoL.
Authors: Ivo Bozovic; Aleksandra Kacar; Stojan Peric; Ana Nikolic; Bogdan Bjelica; Mina Cobeljic; Milutin Petrovic; Aleksandar Stojanov; Vanja Djuric; Miroslav Stojanovic; Gordana Djordjevic; Vesna Martic; Aleksandra Dominovic; Zoran Vukojevic; Ivana Basta Journal: J Neurol Date: 2017-10-30 Impact factor: 4.849
Authors: Jacob N Miller; Alison Kruger; David J Moser; Laurie Gutmann; Ellen van der Plas; Timothy R Koscik; Sarah A Cumming; Darren G Monckton; Peggy C Nopoulos Journal: Front Neurol Date: 2021-07-01 Impact factor: 4.003
Authors: Germán Morís; Libby Wood; Roberto FernáNdez-Torrón; José Andrés González Coraspe; Chris Turner; David Hilton-Jones; Fiona Norwood; Tracey Willis; Matt Parton; Mark Rogers; Simon Hammans; Mark Roberts; Elizabeth Househam; Maggie Williams; Hanns Lochmüller; Teresinha Evangelista Journal: Muscle Nerve Date: 2017-11-07 Impact factor: 3.217