title: Health-related quality of life in ALS, myasthenia gravis and facioscapulohumeral muscular dystrophy.
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.
authors.affiliation: Department of Neurology, Philipps-University Marburg, Rudolf-Bultmannstr. 8, Marburg, Germany.
authors.forename: Yaroslav
authors.initials: Y
authors.lastname: Winter
authors.forename: Karsten
authors.initials: K
authors.lastname: Schepelmann
authors.forename: Annika E
authors.initials: AE
authors.lastname: Spottke
authors.forename: Detlef
authors.initials: D
authors.lastname: Claus
authors.forename: Christoph
authors.initials: C
authors.lastname: Grothe
authors.forename: Rolf
authors.initials: R
authors.lastname: Schröder
authors.forename: Dieter
authors.initials: D
authors.lastname: Heuss
authors.forename: Stefan
authors.initials: S
authors.lastname: Vielhaber
authors.forename: Björn
authors.initials: B
authors.lastname: Tackenberg
authors.forename: Veit
authors.initials: V
authors.lastname: Mylius
authors.forename: Jens-Peter
authors.initials: JP
authors.lastname: Reese
authors.forename: Reinhard
authors.initials: R
authors.lastname: Kiefer
authors.forename: Bertold
authors.initials: B
authors.lastname: Schrank
authors.forename: Wolfgang H
authors.initials: WH
authors.lastname: Oertel
authors.forename: Richard
authors.initials: R
authors.lastname: Dodel
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D015897:Comorbidity; D003430:Cross-Sectional Studies; D005260:Female; D005858:Germany / Q000453:epidemiology; D006304:Health Status; D006801:Humans; D008297:Male; D008875:Middle Aged; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D009157:Myasthenia Gravis / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D011237:Predictive Value of Tests; D011788:Quality of Life / Q000523:psychology
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