Inspiratory Muscle Training in Nemaline Myopathy.
Respiratory muscle weakness
diaphragm
inspiratory muscle
training
nemaline myopathy
Journal
Journal of neuromuscular diseases
ISSN: 2214-3602
Titre abrégé: J Neuromuscul Dis
Pays: Netherlands
ID NLM: 101649948
Informations de publication
Date de publication:
2023
2023
Historique:
medline:
12
9
2023
pubmed:
17
7
2023
entrez:
17
7
2023
Statut:
ppublish
Résumé
Respiratory muscle weakness is a common feature in nemaline myopathy. Inspiratory muscle training (IMT) is an intervention that aims to improve inspiratory muscle strength. The aim of this controlled before-and-after pilot study was to investigate if IMT improves respiratory muscle strength in patients with nemaline myopathy. Nine patients (7 females; 2 males, age 36.6±20.5 years) with respiratory muscle weakness and different clinical phenotypes and genotypes were included. Patients performed eight weeks of sham IMT followed by eight weeks of active threshold IMT. The patients trained twice a day five days a week for 15 minutes at home. The intensity was constant during the training after a gradual increase to 30% of maximal inspiratory pressure (MIP). Active IMT significantly improved MIP from 43±15.9 to 47±16.6 cmH2O (p = 0.019). The effect size was 1.22. There was no significant effect of sham IMT. Sniff nasal inspiratory pressure, maximal expiratory pressure, spirometry, and diaphragm thickness and thickening showed no significant improvements. This pilot study shows that threshold IMT is feasible in patients with nemaline myopathy and improves inspiratory muscle strength. Our findings provide valuable preliminary data for the design of a larger, more comprehensive trial.
Sections du résumé
BACKGROUND
BACKGROUND
Respiratory muscle weakness is a common feature in nemaline myopathy. Inspiratory muscle training (IMT) is an intervention that aims to improve inspiratory muscle strength.
OBJECTIVE
OBJECTIVE
The aim of this controlled before-and-after pilot study was to investigate if IMT improves respiratory muscle strength in patients with nemaline myopathy.
METHODS
METHODS
Nine patients (7 females; 2 males, age 36.6±20.5 years) with respiratory muscle weakness and different clinical phenotypes and genotypes were included. Patients performed eight weeks of sham IMT followed by eight weeks of active threshold IMT. The patients trained twice a day five days a week for 15 minutes at home. The intensity was constant during the training after a gradual increase to 30% of maximal inspiratory pressure (MIP).
RESULTS
RESULTS
Active IMT significantly improved MIP from 43±15.9 to 47±16.6 cmH2O (p = 0.019). The effect size was 1.22. There was no significant effect of sham IMT. Sniff nasal inspiratory pressure, maximal expiratory pressure, spirometry, and diaphragm thickness and thickening showed no significant improvements.
CONCLUSIONS
CONCLUSIONS
This pilot study shows that threshold IMT is feasible in patients with nemaline myopathy and improves inspiratory muscle strength. Our findings provide valuable preliminary data for the design of a larger, more comprehensive trial.
Identifiants
pubmed: 37458044
pii: JND221665
doi: 10.3233/JND-221665
pmc: PMC10578271
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
825-834Références
J Am Coll Cardiol. 2008 Apr 29;51(17):1663-71
pubmed: 18436118
Muscle Nerve. 2013 Jun;47(6):884-9
pubmed: 23625789
J Muscle Res Cell Motil. 2019 Jun;40(2):111-126
pubmed: 31228046
Eur Respir J. 2021 Nov 18;58(5):
pubmed: 33863737
Neuromuscul Disord. 2020 Sep;30(9):750-755
pubmed: 32861531
Can J Neurol Sci. 1998 Aug;25(3):236-41
pubmed: 9706726
Cochrane Database Syst Rev. 2013 Sep 08;(9):CD003792
pubmed: 24014205
Exp Neurol. 2017 Jan;287(Pt 2):216-224
pubmed: 27453480
Clin Rehabil. 2008 Aug;22(8):741-8
pubmed: 18678574
Chest. 1994 Feb;105(2):475-82
pubmed: 8306750
Amyotroph Lateral Scler. 2012 Jan;13(1):33-43
pubmed: 22214352
Pediatr Crit Care Med. 2011 Mar;12(2):e94-8
pubmed: 20407395
Heart Fail Clin. 2015 Jan;11(1):149-72
pubmed: 25432483
Neuromuscul Disord. 2021 Oct;31(10):955-967
pubmed: 34561123
Eur J Heart Fail. 2013 Aug;15(8):892-901
pubmed: 23512093
Mol Genet Metab Rep. 2015 Oct 29;5:67-71
pubmed: 28649546
Cochrane Database Syst Rev. 2019 Sep 05;9:CD011711
pubmed: 31487757
J Appl Physiol (1985). 2018 Aug 1;125(2):381-392
pubmed: 29543134
Neuromuscul Disord. 2002 Jan;12(1):13-8
pubmed: 11731279
Cochrane Database Syst Rev. 2021 Apr 22;4:CD013170
pubmed: 33887060
Eur Respir J. 2011 Feb;37(2):416-25
pubmed: 21282809
Thorax. 1984 Jul;39(7):535-8
pubmed: 6463933
Muscle Nerve. 2010 Dec;42(6):901-7
pubmed: 21104864
Curr Opin Neurol. 2018 Oct;31(5):610-617
pubmed: 30036194
Neuromuscul Disord. 2020 Nov;30(11):904-914
pubmed: 33127291
Semin Pediatr Neurol. 2011 Dec;18(4):230-8
pubmed: 22172418
Clin Rehabil. 2014 Jun;28(6):573-81
pubmed: 24275453
Ann Am Thorac Soc. 2019 Apr;16(4):478-487
pubmed: 30562038
Curr Opin Otolaryngol Head Neck Surg. 2008 Jun;16(3):216-20
pubmed: 18475074
Neuromuscul Disord. 2002 Aug;12(6):576-83
pubmed: 12117483
Thorax. 1993 Jul;48(7):702-7
pubmed: 8153917
Amyotroph Lateral Scler. 2009 Oct-Dec;10(5-6):384-92
pubmed: 19922129
Neuropathology. 2020 Feb;40(1):104-108
pubmed: 31828823
Eur Respir J. 2022 Oct 6;60(4):
pubmed: 35236727
Spine (Phila Pa 1976). 1998 Apr 1;23(7):796-802; discussion 803
pubmed: 9563110
Neurology. 2021 Mar 9;96(10):e1425-e1436
pubmed: 33397769
Am J Respir Crit Care Med. 2002 Aug 15;166(4):518-624
pubmed: 12186831
J Clin Invest. 2020 Feb 3;130(2):754-767
pubmed: 31671076
Neuromuscul Disord. 2006 Aug;16(8):477-80
pubmed: 16793268
Arch Dis Child. 1989 May;64(5):736-8
pubmed: 2658856
Lung. 2016 Aug;194(4):555-61
pubmed: 27106274
Mol Genet Metab. 2016 Feb;117(2):120-8
pubmed: 26381077
Neuromuscul Disord. 1994 May;4(3):249-55
pubmed: 7919973
Front Psychol. 2013 Nov 26;4:863
pubmed: 24324449
J Am Coll Cardiol. 2006 Feb 21;47(4):757-63
pubmed: 16487841
Respir Med. 2008 Dec;102(12):1737-43
pubmed: 18708281
Brain. 2003 Jul;126(Pt 7):1545-51
pubmed: 12805120
J Physiother. 2016 Jul;62(3):138-44
pubmed: 27320833
Neuromuscul Disord. 2018 Mar;28(3):289-298
pubmed: 29395673
Cochrane Database Syst Rev. 2013 Jul 23;(7):CD008507
pubmed: 23881660
Chest. 2005 Sep;128(3):1524-30
pubmed: 16162753
Eur Respir J. 2012 Dec;40(6):1324-43
pubmed: 22743675
J Neurol. 2019 Jan;266(1):133-147
pubmed: 30430231
Front Neurol. 2021 Nov 02;12:761636
pubmed: 34795634
Neuromuscul Disord. 2020 Oct;30(10):866-875
pubmed: 32919842
Am J Respir Crit Care Med. 2019 Oct 15;200(8):e70-e88
pubmed: 31613151
Cochrane Database Syst Rev. 2019 Dec 6;12:CD003907
pubmed: 31808555
Arch Phys Med Rehabil. 2000 Aug;81(8):1065-71
pubmed: 10943756
Dev Med Child Neurol. 1989 Aug;31(4):494-500
pubmed: 2806735
Thorax. 1995 Apr;50(4):371-5
pubmed: 7785009
J Appl Physiol Respir Environ Exerc Physiol. 1983 Jun;54(6):1618-23
pubmed: 6874485
Respiration. 2010;80(5):379-86
pubmed: 19887759
Eur Respir J. 2019 Jun 13;53(6):
pubmed: 30956204