Health-related quality of life in ALS, myasthenia gravis and facioscapulohumeral muscular dystrophy.
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.
Winter
Yaroslav
Y
Department of Neurology, Philipps-University Marburg, Rudolf-Bultmannstr. 8, Marburg, Germany.
Schepelmann
Karsten
K
Spottke
Annika E
AE
Claus
Detlef
D
Grothe
Christoph
C
Schröder
Rolf
R
Heuss
Dieter
D
Vielhaber
Stefan
S
Tackenberg
Björn
B
Mylius
Veit
V
Reese
Jens-Peter
JP
Kiefer
Reinhard
R
Schrank
Bertold
B
Oertel
Wolfgang H
WH
Dodel
Richard
R
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
Neurology. 2006 Dec 26;67(12):2186-91
17190942
J Neurol Sci. 1994 Jul;124 Suppl:96-107
7807156
Neurology. 2001 Feb 27;56(4):442-4
11222784
Amyotroph Lateral Scler. 2007 Dec;8(6):356-61
18033593
Amyotroph Lateral Scler Other Motor Neuron Disord. 2003 Jun;4(2):112-7
14506943
Sleep Med. 2007 Nov;8(7-8):733-41
17512797
J Neurol. 2007 Jul;254(7):931-40
17361345
Qual Life Res. 2004 Oct;13(8):1409-14
15503836
Amyotroph Lateral Scler. 2009 Feb;10(1):15-26
18752087
J Neurol Sci. 2001 Oct 15;191(1-2):87-93
11676997
J Neurol Neurosurg Psychiatry. 2005 Oct;76(10):1406-9
16170086
Arch Phys Med Rehabil. 2005 Jun;86(6):1155-63
15954054
Eur J Neurol. 2006 Feb;13(2):191-3
16490052
J Neurol Neurosurg Psychiatry. 2004 Nov;75(11):1597-601
15489393
J Psychiatr Res. 1975 Nov;12(3):189-98
1202204
Neurol Sci. 2001 Jun;22(3):239-45
11731877
Neurology. 2000 Jul 12;55(1):16-23
10891897
Parkinsonism Relat Disord. 2010 May;16(4):243-8
20022549
Parkinsonism Relat Disord. 2005 Jun;11(4):221-6
15878582
Sports Med. 2000 Mar;29(3):167-80
10739267
Neuroepidemiology. 1989;8(3):142-50
2725806
Gesundheitswesen. 1999 Dec;61 Spec No:S184-90
10726419
Gesundheitswesen. 2005 Mar;67(3):173-82
15789280
Neurology. 2003 Mar 11;60(5):813-9
12629239
J Adv Nurs. 2000 Jul;32(1):164-73
10886448
Muscle Nerve. 2009 Aug;40(2):200-5
19609906
Qual Life Res. 1993 Jun;2(3):169-80
8401453
Mov Disord. 2009 Mar 15;24(4):574-8
19097181
Neurology. 2005 Oct 25;65(8):1264-7
16247055
Am J Hosp Palliat Care. 2002 Jan-Feb;19(1):39-48
12173612
J Neurol. 2000 Nov;247(11):835-40
11151414
J Neurol. 2010 Apr;257(4):638-45
19946784
J Neurol Neurosurg Psychiatry. 2000 Sep;69(3):308-12
10945804
Neurosci Biobehav Rev. 2001 Jan;25(1):75-81
11166079
Neurol Sci. 2010 Feb;31(1):41-5
19816655
Neurol Sci. 2001 Oct;22(5):363-9
11917973
J Neurol. 2010 Jan;257(1):15-23
19629566
Amyotroph Lateral Scler. 2007 Aug;8(4):243-54
17653923
Muscle Nerve. 2008 Aug;38(2):947-56
18697209
Arch Phys Med Rehabil. 1957 Sep;38(9):574-9
13459572
Parkinsonism Relat Disord. 2007 Mar;13(2):108-14
17055326
Neurology. 1999 Jun 10;52(9):1822-6
10371529
J Neurol. 2007 Jul;254(7):948-9
17325819
Eur J Health Econ. 2005 Jun;6(2):124-30
19787848