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Respiratory paralysis

MedGen UID:
19748
Concept ID:
C0035232
Finding
Synonyms: Diaphragmatic Paralysis; Muscle Paralyses, Respiratory; Muscle Paralysis, Respiratory; Paralysis, Diaphragmatic; Paralysis, Respiratory; Paralysis, Respiratory Muscle; Respiratory Muscle Paralysis; Respiratory Paralysis
 
HPO: HP:0002203
Monarch Initiative: MONDO:0043775

Definition

Inability to move the muscles of respiration. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRespiratory paralysis

Conditions with this feature

Hereditary coproporphyria
MedGen UID:
57931
Concept ID:
C0162531
Disease or Syndrome
Hereditary coproporphyria (HCP) is an acute (hepatic) porphyria in which the acute symptoms are neurovisceral and occur in discrete episodes. Attacks typically start in the abdomen with low-grade pain that slowly increases over a period of days (not hours) with nausea progressing to vomiting. In some individuals, the pain is predominantly in the back or extremities. When an acute attack is untreated, a motor neuropathy may develop over a period of days or a few weeks. The neuropathy first appears as weakness proximally in the arms and legs, then progresses distally to involve the hands and feet. Some individuals experience respiratory insufficiency due to loss of innervation of the diaphragm and muscles of respiration. Acute attacks are associated commonly with use of certain medications, caloric deprivation, and changes in female reproductive hormones. About 20% of those with an acute attack also experience photosensitivity associated with bullae and skin fragility.
Acute intermittent porphyria
MedGen UID:
56452
Concept ID:
C0162565
Disease or Syndrome
Acute intermittent porphyria (AIP), an autosomal dominant disorder, occurs in heterozygotes for an HMBS pathogenic variant that causes reduced activity of the enzyme porphobilinogen deaminase. AIP is considered "overt" in a heterozygote who was previously or is currently symptomatic; AIP is considered "latent" in a heterozygote who has never had symptoms, and typically has been identified during molecular genetic testing of at-risk family members. Note that GeneReviews does not use the term "carrier" for an individual who is heterozygous for an autosomal dominant pathogenic variant; GeneReviews reserves the term "carrier" for an individual who is heterozygous for an autosomal recessive disorder and thus is not expected to ever develop manifestations of the disorder. Overt AIP is characterized clinically by life-threatening acute neurovisceral attacks of severe abdominal pain without peritoneal signs, often accompanied by nausea, vomiting, tachycardia, and hypertension. Attacks may be complicated by neurologic findings (mental changes, convulsions, and peripheral neuropathy that may progress to respiratory paralysis), and hyponatremia. Acute attacks, which may be provoked by certain drugs, alcoholic beverages, endocrine factors, calorie restriction, stress, and infections, usually resolve within two weeks. Most individuals with AIP have one or a few attacks; about 3%-8% (mainly women) have recurrent attacks (defined as >3 attacks/year) that may persist for years. Other long-term complications are chronic renal failure, hepatocellular carcinoma (HCC), and hypertension. Attacks, which are very rare before puberty, are more common in women than men. Latent AIP. While all individuals heterozygous for an HMBS pathogenic variant that predisposes to AIP are at risk of developing overt AIP, most have latent AIP and never have symptoms.
Porphobilinogen synthase deficiency
MedGen UID:
78659
Concept ID:
C0268328
Disease or Syndrome
ALAD porphyria is a rare autosomal recessive disorder that has been reported and confirmed by genetic analysis in only 5 patients (Jaffe and Stith, 2007).
Muscular atrophy, malignant neurogenic
MedGen UID:
322469
Concept ID:
C1834689
Disease or Syndrome

Professional guidelines

PubMed

Kim D, Kim S, Sung A, Patel N, Wong N, Conboy MJ, Conboy IM
Transl Neurodegener 2022 Mar 11;11(1):16. doi: 10.1186/s40035-022-00290-5. PMID: 35272709Free PMC Article
Gorchein A
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Recent clinical studies

Etiology

Boentert M, Cao M, Mass D, De Mattia E, Falcier E, Goncalves M, Holland V, Katz SL, Orlikowski D, Sannicolò G, Wijkstra P, Hellerstein L, Sansone VA
Respiration 2020;99(4):360-368. Epub 2020 Apr 16 doi: 10.1159/000505634. PMID: 32299079
Corbett B, Clark RF
Emerg Med Clin North Am 2017 May;35(2):339-354. doi: 10.1016/j.emc.2016.12.003. PMID: 28411931
Gaissert H, Wilcox SR
Thorac Cardiovasc Surg 2016 Dec;64(8):621-630. Epub 2016 Nov 26 doi: 10.1055/s-0036-1595816. PMID: 27888814
Berlowitz DJ, Tamplin J
Cochrane Database Syst Rev 2013 Jul 23;(7):CD008507. doi: 10.1002/14651858.CD008507.pub2. PMID: 23881660
Goel A, Aggarwal P
Natl Med J India 2007 Jul-Aug;20(4):182-91. PMID: 18085124

Diagnosis

Kilaru D, Panebianco N, Baston C
Chest 2021 Mar;159(3):1166-1172. Epub 2020 Dec 10 doi: 10.1016/j.chest.2020.12.003. PMID: 33309837
Ricoy J, Rodríguez-Núñez N, Álvarez-Dobaño JM, Toubes ME, Riveiro V, Valdés L
Pulmonology 2019 Jul-Aug;25(4):223-235. Epub 2018 Dec 1 doi: 10.1016/j.pulmoe.2018.10.008. PMID: 30509855
Nason LK, Walker CM, McNeeley MF, Burivong W, Fligner CL, Godwin JD
Radiographics 2012 Mar-Apr;32(2):E51-70. doi: 10.1148/rg.322115127. PMID: 22411950
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
American Thoracic Society/European Respiratory Society
Am J Respir Crit Care Med 2002 Aug 15;166(4):518-624. doi: 10.1164/rccm.166.4.518. PMID: 12186831

Therapy

Kilaru D, Panebianco N, Baston C
Chest 2021 Mar;159(3):1166-1172. Epub 2020 Dec 10 doi: 10.1016/j.chest.2020.12.003. PMID: 33309837
Corbett B, Clark RF
Emerg Med Clin North Am 2017 May;35(2):339-354. doi: 10.1016/j.emc.2016.12.003. PMID: 28411931
Gaissert H
Thorac Cardiovasc Surg 2016 Dec;64(8):620. Epub 2016 Dec 13 doi: 10.1055/s-0036-1595813. PMID: 27960202
Lu JF, Nightingale CH
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Prognosis

Caggiano S, Khirani S, Dabaj I, Cavassa E, Amaddeo A, Arroyo JO, Desguerre I, Richard P, Cutrera R, Ferreiro A, Estournet B, Quijano-Roy S, Fauroux B
Neuromuscul Disord 2017 Aug;27(8):747-755. Epub 2017 Apr 26 doi: 10.1016/j.nmd.2017.04.010. PMID: 28606403
Rabinstein AA
Continuum (Minneap Minn) 2015 Oct;21(5 Neurocritical Care):1324-45. doi: 10.1212/CON.0000000000000218. PMID: 26426233
Sarwal A, Walker FO, Cartwright MS
Muscle Nerve 2013 Mar;47(3):319-29. Epub 2013 Feb 4 doi: 10.1002/mus.23671. PMID: 23382111Free PMC Article
Qureshi A
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Chervin RD, Guilleminault C
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Clinical prediction guides

Hiramatsu S, Moriwaki K, Nakao M, Tsutsumi YM
Can J Anaesth 2022 Mar;69(3):364-368. Epub 2021 Dec 13 doi: 10.1007/s12630-021-02168-y. PMID: 34902106Free PMC Article
Ricoy J, Rodríguez-Núñez N, Álvarez-Dobaño JM, Toubes ME, Riveiro V, Valdés L
Pulmonology 2019 Jul-Aug;25(4):223-235. Epub 2018 Dec 1 doi: 10.1016/j.pulmoe.2018.10.008. PMID: 30509855
Berlowitz DJ, Tamplin J
Cochrane Database Syst Rev 2013 Jul 23;(7):CD008507. doi: 10.1002/14651858.CD008507.pub2. PMID: 23881660
Decramer M
Verh K Acad Geneeskd Belg 2001;63(6):577-602; discussion 602-4. PMID: 11813511
Gorchein A
Br J Clin Pharmacol 1997 Nov;44(5):427-34. doi: 10.1046/j.1365-2125.1997.t01-1-00609.x. PMID: 9384458Free PMC Article

Recent systematic reviews

Yekzaman BR, Minchew HM, Alvarado A, Ohiorhenuan I
World Neurosurg 2022 Nov;167:74-77. Epub 2022 Sep 8 doi: 10.1016/j.wneu.2022.09.009. PMID: 36089276
Pappal RD, Roberts BW, Winkler W, Yaegar LH, Stephens RJ, Fuller BM
Crit Care Med 2021 Mar 1;49(3):e304-e314. doi: 10.1097/CCM.0000000000004824. PMID: 33566462Free PMC Article
O'Connell JS, Janssen Lok M, Miyake H, Seo S, Bindi E, Alganabi M, Pierro A
Pediatr Surg Int 2019 Jan;35(1):87-95. Epub 2018 Nov 10 doi: 10.1007/s00383-018-4379-1. PMID: 30415438
Berlowitz DJ, Tamplin J
Cochrane Database Syst Rev 2013 Jul 23;(7):CD008507. doi: 10.1002/14651858.CD008507.pub2. PMID: 23881660
López-Arrieta JM, Schneider L
Cochrane Database Syst Rev 2006 Apr 19;(2):CD003155. doi: 10.1002/14651858.CD003155.pub3. PMID: 16625573

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