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Health-related quality of life in ALS, myasthenia gravis and facioscapulohumeral muscular dystrophy

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Abstract

Neuromuscular disorders are rare diseases with a chronic and debilitating course. Unfortunately, data on the health-related quality of life (HRQoL) in neuromuscular diseases are limited. The objective of this multicentre cross-sectional study was to compare the HRQoL in patients with amyotrophic lateral sclerosis (ALS), facioscapulohumeral muscular dystrophy (FSHD) and myasthenia gravis (MG) and to identify the determinants of the HRQoL in these diseases. We recruited 91 consecutive outpatients with ALS (n = 37), FSHD (n = 17) or MG (n = 37) in seven specialized German health centres. The HRQoL was determined using the 36-Item Short Form Health Survey (SF-36) and the EuroQol (EQ-5D). Independent predictors of the HRQoL were identified using multiple regression analysis. The HRQoL in all domains of the SF-36, except for bodily pain, was significantly reduced. The domains related to physical health (physical functioning, physical role) were most affected. The EQ-5D-index score was most reduced in ALS (0.54) and least reduced in MG (0.89). Independent predictors of a reduced HRQoL were disease severity and depression in ALS, and disease severity, depression, older age and increased body-mass index in MG. The patterns of HRQoL-impairment in neuromuscular disorders share some common features, such as a more pronounced reduction in the HRQoL related to physical health, but there are a number of disease-specific features that should be considered in outcomes of clinical trials and treatment guidelines. In addition to the treatment of motor symptoms, greater attention should be paid to the treatment of depression, which was found to be among the independent predictors of the HRQoL in ALS and MG.

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Acknowledgment

This study was sponsored by the German Society for Muscle Disorders (Deutsche Gesellschaft für Muskelkranke) and the BMBF (project no. 01GI9901/1). We would like to thank Mrs. Jennifer Manne for language editing. The corresponding author had full access to all the data in the study.

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Authors and Affiliations

  1. Department of Neurology, Philipps-University Marburg, Rudolf-Bultmannstr. 8, 35039, Marburg, Germany

    Yaroslav Winter, Karsten Schepelmann, Björn Tackenberg, Veit Mylius, Jens-Peter Reese, Wolfgang H. Oertel & Richard Dodel

  2. Department of Neurology, Friedrich-Wilhelms-University, Bonn, Germany

    Annika E. Spottke, Christoph Grothe & Rolf Schröder

  3. Department of Neurology, Klinikum Darmstadt, Darmstadt, Germany

    Detlef Claus

  4. Department of Neurology, Friedrich-Alexander University Erlangen, Erlangen, Germany

    Dieter Heuss

  5. Department of Neurology, Otto-von-Guericke University Magdeburg, Magdeburg, Germany

    Stefan Vielhaber

  6. Department of Neurology, University of Muenster, Muenster, Germany

    Reinhard Kiefer

  7. Department of Neurology, Clinic of Diagnostics, Wiesbaden, Germany

    Bertold Schrank

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  1. Yaroslav Winter
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  2. Karsten Schepelmann
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  14. Wolfgang H. Oertel
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  15. Richard Dodel
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Correspondence to Richard Dodel.

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Winter, Y., Schepelmann, K., Spottke, A.E. et al. Health-related quality of life in ALS, myasthenia gravis and facioscapulohumeral muscular dystrophy. J Neurol 257, 1473–1481 (2010). https://doi.org/10.1007/s00415-010-5549-9

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  • DOI: https://doi.org/10.1007/s00415-010-5549-9

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