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Prospective measurement of quality of life in myotonic dystrophy type 1
Author(s) -
Peric S.,
Heatwole C.,
Durovic E.,
Kacar A.,
Nikolic A.,
Basta I.,
Marjanovic A.,
Stevic Z.,
Lavrnic D.,
Rakocevic Stojanovic V.
Publication year - 2017
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/ane.12788
Subject(s) - medicine , myotonic dystrophy , quality of life (healthcare) , physical therapy , rating scale , outpatient clinic , cohort , muscular dystrophy , neurology , prospective cohort study , functional independence measure , myotonia , activities of daily living , weakness , psychology , surgery , psychiatry , developmental psychology , nursing
Generic patient reported outcome measures have had varied success in tracking QoL in myotonic dystrophy type 1 ( DM 1). Aim To analyze changes of Individualized Neuromuscular Quality of Life questionnaire ( INQ oL) scores in clinic patients with DM 1 over a 6‐year period. Method Patients completed the INQ oL at baseline and after a 6‐year period through their attendance in a neurology outpatient clinic. Severity of muscular involvement in DM 1 was analyzed using the Muscular Impairment Rating Scale ( MIRS ). Results Ninety‐nine DM 1 patients completed a baseline visit. Sixty‐seven of these patients were retested at an interval time. The overall INQ oL score improved in our sample of patients ( P <.05) as did the following subscales: myotonia ( P <.05), pain ( P <.05), activities ( P <.01), social relationships ( P <.01), and body image ( P <.05). No changes were observed for the independence and emotions scales. There were no differences in mean change of INQ oL scores between patients with worsened MIRS and those with no change in MIRS scale after follow‐up ( P >.05). Conclusion Individualized Neuromuscular Quality of Life questionnaire scores improved in our cohort of DM 1 patients during a 6‐year period. INQ oL score did not correlate with progression of muscle weakness. This must be better understood before the selection of the instrument for use in trials to measure therapeutic benefit in DM 1 patients.