Health-Related Quality of Life in Patients with Adult-Onset Myotonic Dystrophy Type 1: A Systematic Review

Erik Landfeldt (), Josefin Edström, Cecilia Jimenez-Moreno, Baziel G. M. Engelen, Janbernd Kirschner and Hanns Lochmüller
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Erik Landfeldt: Institute of Environmental Medicine, Karolinska Institutet
Josefin Edström: Saco
Cecilia Jimenez-Moreno: Newcastle University
Baziel G. M. Engelen: Radboud University Medical Center
Janbernd Kirschner: University of Freiburg, Faculty of Medicine
Hanns Lochmüller: University of Freiburg, Faculty of Medicine

The Patient: Patient-Centered Outcomes Research, 2019, vol. 12, issue 4, No 2, 365-373

Abstract: Abstract Background Adult-onset myotonic dystrophy type 1 (DM1) is a chronic, multisystem disorder that leads to disability and premature death. Objectives The objective of our study was to conduct a systematic literature review of the health-related quality of life (HRQoL) of patients with DM1. Methods We searched Embase, Web of Science, and PubMed for English language full-text articles reporting results from studies of HRQoL in patients with adult-onset DM1 published between 1 January 2000 and 21 February 2018. We excluded reviews, editorials, and studies reporting results for a sample with fewer than five patients (to allow for meaningful inference). Results The search identified 266 unique publications. Of these, 231 were excluded following title and abstract screening and 16 after full-text review, leaving 19 articles for data synthesis. We found 15 articles measuring the HRQoL of patients with adult-onset DM1 using the 36-Item Short Form Health Survey (SF-36), six using the Individualized Neuromuscular Quality of Life Questionnaire (INQoL), and one using Cantril’s Ladder. Available evidence shows that patient HRQoL is impaired in DM1, mainly due to compromised physical health, but also reveals that substantial heterogeneity exists in estimates across studies. Conclusions HRQoL in adult-onset DM1 has been extensively studied using the SF-36 and the INQoL, but current estimates are inconclusive, and little is known of the impact of the disease as measured using other instruments. Our data synthesis should help characterize the patient burden of DM1 and inform future studies of HRQoL in this indication.

Date: 2019
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DOI: 10.1007/s40271-019-00357-y

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