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Respiratory Failure Related to Neuromuscular Disorder

MedGen UID:
768729
•Concept ID:
C3640089
•
Disease or Syndrome
Synonym: Respiratory Failure due to Neuromuscular Disorder

Definition

Impaired gas exchange by the respiratory system resulting in hypoxemia and decreased oxygenation of the tissues that may be associated with increased arterial levels of carbon dioxide; the respiratory failure is due to a neuromuscular disorder. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRespiratory Failure Related to Neuromuscular Disorder

Professional guidelines

PubMed

Clinical Guidelines for Diagnosis and Treatment of Botulism, 2021.
Rao AK, Sobel J, Chatham-Stephens K, Luquez C
MMWR Recomm Rep 2021 May 7;70(2):1-30. doi: 10.15585/mmwr.rr7002a1. PMID: 33956777Free PMC Article
Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis.
van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA
Nat Rev Neurol 2014 Aug;10(8):469-82. Epub 2014 Jul 15 doi: 10.1038/nrneurol.2014.121. PMID: 25023340
Consensus statement for standard of care in spinal muscular atrophy.
Wang CH, Finkel RS, Bertini ES, Schroth M, Simonds A, Wong B, Aloysius A, Morrison L, Main M, Crawford TO, Trela A; Participants of the International Conference on SMA Standard of Care
J Child Neurol 2007 Aug;22(8):1027-49. doi: 10.1177/0883073807305788. PMID: 17761659

Recent clinical studies

Etiology

Respiratory Management of Patients With Neuromuscular Weakness: An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report.
Khan A, Frazer-Green L, Amin R, Wolfe L, Faulkner G, Casey K, Sharma G, Selim B, Zielinski D, Aboussouan LS, McKim D, Gay P
Chest 2023 Aug;164(2):394-413. Epub 2023 Mar 13 doi: 10.1016/j.chest.2023.03.011. PMID: 36921894
Treatment for sialorrhea (excessive saliva) in people with motor neuron disease/amyotrophic lateral sclerosis.
James E, Ellis C, Brassington R, Sathasivam S, Young CA
Cochrane Database Syst Rev 2022 May 20;5(5):CD006981. doi: 10.1002/14651858.CD006981.pub3. PMID: 35593746Free PMC Article
Phase 1-2 Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS.
Miller T, Cudkowicz M, Shaw PJ, Andersen PM, Atassi N, Bucelli RC, Genge A, Glass J, Ladha S, Ludolph AL, Maragakis NJ, McDermott CJ, Pestronk A, Ravits J, Salachas F, Trudell R, Van Damme P, Zinman L, Bennett CF, Lane R, Sandrock A, Runz H, Graham D, Houshyar H, McCampbell A, Nestorov I, Chang I, McNeill M, Fanning L, Fradette S, Ferguson TA
N Engl J Med 2020 Jul 9;383(2):109-119. doi: 10.1056/NEJMoa2003715. PMID: 32640130
Myasthenia gravis.
Gilhus NE, Tzartos S, Evoli A, Palace J, Burns TM, Verschuuren JJGM
Nat Rev Dis Primers 2019 May 2;5(1):30. doi: 10.1038/s41572-019-0079-y. PMID: 31048702
Long-term complications of critical care.
Desai SV, Law TJ, Needham DM
Crit Care Med 2011 Feb;39(2):371-9. doi: 10.1097/CCM.0b013e3181fd66e5. PMID: 20959786

Diagnosis

Clinical Guidelines for Diagnosis and Treatment of Botulism, 2021.
Rao AK, Sobel J, Chatham-Stephens K, Luquez C
MMWR Recomm Rep 2021 May 7;70(2):1-30. doi: 10.15585/mmwr.rr7002a1. PMID: 33956777Free PMC Article
Amyotrophic lateral sclerosis: a clinical review.
Masrori P, Van Damme P
Eur J Neurol 2020 Oct;27(10):1918-1929. Epub 2020 Jul 7 doi: 10.1111/ene.14393. PMID: 32526057Free PMC Article
Myasthenia Gravis and Congenital Myasthenic Syndromes.
Ciafaloni E
Continuum (Minneap Minn) 2019 Dec;25(6):1767-1784. doi: 10.1212/CON.0000000000000800. PMID: 31794470
Myasthenia gravis.
Gilhus NE, Tzartos S, Evoli A, Palace J, Burns TM, Verschuuren JJGM
Nat Rev Dis Primers 2019 May 2;5(1):30. doi: 10.1038/s41572-019-0079-y. PMID: 31048702
Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis.
van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA
Nat Rev Neurol 2014 Aug;10(8):469-82. Epub 2014 Jul 15 doi: 10.1038/nrneurol.2014.121. PMID: 25023340

Therapy

Abatacept/Ruxolitinib and Screening for Concomitant Respiratory Muscle Failure to Mitigate Fatality of Immune-Checkpoint Inhibitor Myocarditis.
Salem JE, Bretagne M, Abbar B, Leonard-Louis S, Ederhy S, Redheuil A, Boussouar S, Nguyen LS, Procureur A, Stein F, Fenioux C, Devos P, Gougis P, Dres M, Demoule A, Psimaras D, Lenglet T, Maisonobe T, De Chambrun MP, Hekimian G, Straus C, Gonzalez-Bermejo J, Klatzmann D, Rigolet A, Guillaume-Jugnot P, Champtiaux N, Benveniste O, Weiss N, Saheb S, Rouvier P, Plu I, Gandjbakhch E, Kerneis M, Hammoudi N, Zahr N, Llontop C, Morelot-Panzini C, Lehmann L, Qin J, Moslehi JJ, Rosenzwajg M, Similowski T, Allenbach Y
Cancer Discov 2023 May 4;13(5):1100-1115. doi: 10.1158/2159-8290.CD-22-1180. PMID: 36815259
Treatment for sialorrhea (excessive saliva) in people with motor neuron disease/amyotrophic lateral sclerosis.
James E, Ellis C, Brassington R, Sathasivam S, Young CA
Cochrane Database Syst Rev 2022 May 20;5(5):CD006981. doi: 10.1002/14651858.CD006981.pub3. PMID: 35593746Free PMC Article
Phase 1-2 Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS.
Miller T, Cudkowicz M, Shaw PJ, Andersen PM, Atassi N, Bucelli RC, Genge A, Glass J, Ladha S, Ludolph AL, Maragakis NJ, McDermott CJ, Pestronk A, Ravits J, Salachas F, Trudell R, Van Damme P, Zinman L, Bennett CF, Lane R, Sandrock A, Runz H, Graham D, Houshyar H, McCampbell A, Nestorov I, Chang I, McNeill M, Fanning L, Fradette S, Ferguson TA
N Engl J Med 2020 Jul 9;383(2):109-119. doi: 10.1056/NEJMoa2003715. PMID: 32640130
Myasthenia Gravis and Congenital Myasthenic Syndromes.
Ciafaloni E
Continuum (Minneap Minn) 2019 Dec;25(6):1767-1784. doi: 10.1212/CON.0000000000000800. PMID: 31794470
Myasthenia gravis.
Gilhus NE, Tzartos S, Evoli A, Palace J, Burns TM, Verschuuren JJGM
Nat Rev Dis Primers 2019 May 2;5(1):30. doi: 10.1038/s41572-019-0079-y. PMID: 31048702

Prognosis

Current State and Future Directions in the Diagnosis of Amyotrophic Lateral Sclerosis.
Vidovic M, Müschen LH, Brakemeier S, Machetanz G, Naumann M, Castro-Gomez S
Cells 2023 Feb 24;12(5) doi: 10.3390/cells12050736. PMID: 36899872Free PMC Article
Mechanical ventilation in Guillain-Barré syndrome.
Shang P, Zhu M, Baker M, Feng J, Zhou C, Zhang HL
Expert Rev Clin Immunol 2020 Nov;16(11):1053-1064. Epub 2020 Nov 25 doi: 10.1080/1744666X.2021.1840355. PMID: 33112177
Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis.
van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA
Nat Rev Neurol 2014 Aug;10(8):469-82. Epub 2014 Jul 15 doi: 10.1038/nrneurol.2014.121. PMID: 25023340
Long-term complications of critical care.
Desai SV, Law TJ, Needham DM
Crit Care Med 2011 Feb;39(2):371-9. doi: 10.1097/CCM.0b013e3181fd66e5. PMID: 20959786
Amyotrophic lateral sclerosis.
Wijesekera LC, Leigh PN
Orphanet J Rare Dis 2009 Feb 3;4:3. doi: 10.1186/1750-1172-4-3. PMID: 19192301Free PMC Article

Clinical prediction guides

Long-COVID and Post-COVID Health Complications: An Up-to-Date Review on Clinical Conditions and Their Possible Molecular Mechanisms.
Silva Andrade B, Siqueira S, de Assis Soares WR, de Souza Rangel F, Santos NO, Dos Santos Freitas A, Ribeiro da Silveira P, Tiwari S, Alzahrani KJ, Góes-Neto A, Azevedo V, Ghosh P, Barh D
Viruses 2021 Apr 18;13(4) doi: 10.3390/v13040700. PMID: 33919537Free PMC Article
Mechanical ventilation in Guillain-Barré syndrome.
Shang P, Zhu M, Baker M, Feng J, Zhou C, Zhang HL
Expert Rev Clin Immunol 2020 Nov;16(11):1053-1064. Epub 2020 Nov 25 doi: 10.1080/1744666X.2021.1840355. PMID: 33112177
Phase 1-2 Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS.
Miller T, Cudkowicz M, Shaw PJ, Andersen PM, Atassi N, Bucelli RC, Genge A, Glass J, Ladha S, Ludolph AL, Maragakis NJ, McDermott CJ, Pestronk A, Ravits J, Salachas F, Trudell R, Van Damme P, Zinman L, Bennett CF, Lane R, Sandrock A, Runz H, Graham D, Houshyar H, McCampbell A, Nestorov I, Chang I, McNeill M, Fanning L, Fradette S, Ferguson TA
N Engl J Med 2020 Jul 9;383(2):109-119. doi: 10.1056/NEJMoa2003715. PMID: 32640130
Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis.
van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA
Nat Rev Neurol 2014 Aug;10(8):469-82. Epub 2014 Jul 15 doi: 10.1038/nrneurol.2014.121. PMID: 25023340
Long-term complications of critical care.
Desai SV, Law TJ, Needham DM
Crit Care Med 2011 Feb;39(2):371-9. doi: 10.1097/CCM.0b013e3181fd66e5. PMID: 20959786

Recent systematic reviews

Respiratory Management of Patients With Neuromuscular Weakness: An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report.
Khan A, Frazer-Green L, Amin R, Wolfe L, Faulkner G, Casey K, Sharma G, Selim B, Zielinski D, Aboussouan LS, McKim D, Gay P
Chest 2023 Aug;164(2):394-413. Epub 2023 Mar 13 doi: 10.1016/j.chest.2023.03.011. PMID: 36921894
Neuromuscular and Neuromuscular Junction Manifestations of the PURA-NDD: A Systematic Review of the Reported Symptoms and Potential Treatment Options.
Mroczek M, Iyadurai S
Int J Mol Sci 2023 Jan 23;24(3) doi: 10.3390/ijms24032260. PMID: 36768582Free PMC Article
Treatment for sialorrhea (excessive saliva) in people with motor neuron disease/amyotrophic lateral sclerosis.
James E, Ellis C, Brassington R, Sathasivam S, Young CA
Cochrane Database Syst Rev 2022 May 20;5(5):CD006981. doi: 10.1002/14651858.CD006981.pub3. PMID: 35593746Free PMC Article
Immune checkpoint inhibitor related myasthenia gravis: single center experience and systematic review of the literature.
Safa H, Johnson DH, Trinh VA, Rodgers TE, Lin H, Suarez-Almazor ME, Fa'ak F, Saberian C, Yee C, Davies MA, Tummala S, Woodman K, Abdel-Wahab N, Diab A
J Immunother Cancer 2019 Nov 21;7(1):319. doi: 10.1186/s40425-019-0774-y. PMID: 31753014Free PMC Article
Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease.
Ng L, Khan F, Young CA, Galea M
Cochrane Database Syst Rev 2017 Jan 10;1(1):CD011776. doi: 10.1002/14651858.CD011776.pub2. PMID: 28072907Free PMC Article

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