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Acute motor axonal neuropathy

MedGen UID:
855842
•Concept ID:
C3890941
•
Disease or Syndrome
Synonyms: Acute Motor Axonal Neuropathy; acute motor axonal neuropathy; Acute pure motor GBS; acute pure motor GBS; acute pure motor Guillain-Barre syndrome; Acute pure motor Guillain-Barré syndrome; acute pure motor Guillain-Barré syndrome; AMAN
SNOMED CT: Acute motor axonal neuropathy (715770009); Acute pure motor Guillain-Barré syndrome (715770009)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0020349
Orphanet: ORPHA98918

Definition

A pure motor axonal form of Guillain-Barré syndrome that presents with rapid onset of muscle weakness and absent reflexes. The clinical course tends to be more severe than in the more frequent, demyelinating form of Guillain-Barré syndrome. In the majority of cases, this disease occurs following Campylobacter jejuni infection, in particular following infection with strains of C jejuni that cause enteritis. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute motor axonal neuropathy

Professional guidelines

PubMed

Diagnosis of Guillain-Barré syndrome and validation of the Brighton criteria in Malaysia.
Tan CY, Razali SNO, Goh KJ, Shahrizaila N
J Peripher Nerv Syst 2020 Sep;25(3):256-264. Epub 2020 Jun 29 doi: 10.1111/jns.12398. PMID: 32511817
Diagnosis, treatment and prognosis of Guillain-Barré syndrome (GBS).
van Doorn PA
Presse Med 2013 Jun;42(6 Pt 2):e193-201. Epub 2013 Apr 28 doi: 10.1016/j.lpm.2013.02.328. PMID: 23628447
Guillain-Barré syndrome: epidemiology, pathophysiology and management.
Kuwabara S
Drugs 2004;64(6):597-610. doi: 10.2165/00003495-200464060-00003. PMID: 15018590

Recent clinical studies

Etiology

Pathology explains various mechanisms of auto-immune inflammatory peripheral neuropathies.
Vallat JM, Mathis S
Brain Pathol 2024 Mar;34(2):e13184. Epub 2023 Jun 25 doi: 10.1111/bpa.13184. PMID: 37356965Free PMC Article
Clinical and Electrophysiological Features of Chronic Motor Axonal Neuropathy.
Zhang D, Geng H, Cao L, Li W
J Clin Neurophysiol 2022 May 1;39(4):317-323. Epub 2020 Nov 20 doi: 10.1097/WNP.0000000000000771. PMID: 32852287
Pain in acute motor axonal neuropathy.
Zhao F, Wang J, Zhang J, Pang X, Huang S, Chang X, Guo J, Zhang W
Muscle Nerve 2021 Dec;64(6):739-743. Epub 2021 Sep 24 doi: 10.1002/mus.27414. PMID: 34506042
Guillain-Barré syndrome, transverse myelitis and infectious diseases.
Rodríguez Y, Rojas M, Pacheco Y, Acosta-Ampudia Y, Ramírez-Santana C, Monsalve DM, Gershwin ME, Anaya JM
Cell Mol Immunol 2018 Jun;15(6):547-562. Epub 2018 Jan 29 doi: 10.1038/cmi.2017.142. PMID: 29375121Free PMC Article
Guillain-Barré syndrome.
Ansar V, Valadi N
Prim Care 2015 Jun;42(2):189-93. Epub 2015 Mar 20 doi: 10.1016/j.pop.2015.01.001. PMID: 25979580

Diagnosis

Axonal variants of Guillain-Barré syndrome: an update.
Shang P, Zhu M, Wang Y, Zheng X, Wu X, Zhu J, Feng J, Zhang HL
J Neurol 2021 Jul;268(7):2402-2419. Epub 2020 Mar 5 doi: 10.1007/s00415-020-09742-2. PMID: 32140865
Guillain-Barré syndrome.
Esposito S, Longo MR
Autoimmun Rev 2017 Jan;16(1):96-101. Epub 2016 Sep 23 doi: 10.1016/j.autrev.2016.09.022. PMID: 27666816
Diagnosis, treatment and prognosis of Guillain-Barré syndrome (GBS).
van Doorn PA
Presse Med 2013 Jun;42(6 Pt 2):e193-201. Epub 2013 Apr 28 doi: 10.1016/j.lpm.2013.02.328. PMID: 23628447
Guillain-Barré syndrome.
Arcila-Londono X, Lewis RA
Semin Neurol 2012 Jul;32(3):179-86. Epub 2012 Nov 1 doi: 10.1055/s-0032-1329196. PMID: 23117942
Guillain-Barré syndrome.
Hughes RA, Cornblath DR
Lancet 2005 Nov 5;366(9497):1653-66. doi: 10.1016/S0140-6736(05)67665-9. PMID: 16271648

Therapy

Axonal variants of Guillain-Barré syndrome: an update.
Shang P, Zhu M, Wang Y, Zheng X, Wu X, Zhu J, Feng J, Zhang HL
J Neurol 2021 Jul;268(7):2402-2419. Epub 2020 Mar 5 doi: 10.1007/s00415-020-09742-2. PMID: 32140865
Guillain-Barré syndrome.
Esposito S, Longo MR
Autoimmun Rev 2017 Jan;16(1):96-101. Epub 2016 Sep 23 doi: 10.1016/j.autrev.2016.09.022. PMID: 27666816
Guillain-Barré syndrome.
Ansar V, Valadi N
Prim Care 2015 Jun;42(2):189-93. Epub 2015 Mar 20 doi: 10.1016/j.pop.2015.01.001. PMID: 25979580
Guillain-Barré syndrome.
Hughes RA, Cornblath DR
Lancet 2005 Nov 5;366(9497):1653-66. doi: 10.1016/S0140-6736(05)67665-9. PMID: 16271648
Guillain-Barré syndrome: epidemiology, pathophysiology and management.
Kuwabara S
Drugs 2004;64(6):597-610. doi: 10.2165/00003495-200464060-00003. PMID: 15018590

Prognosis

Clinical and Electrophysiological Features of Chronic Motor Axonal Neuropathy.
Zhang D, Geng H, Cao L, Li W
J Clin Neurophysiol 2022 May 1;39(4):317-323. Epub 2020 Nov 20 doi: 10.1097/WNP.0000000000000771. PMID: 32852287
The immunotherapy of Guillain-Barré syndrome.
Restrepo-Jiménez P, Rodríguez Y, González P, Chang C, Gershwin ME, Anaya JM
Expert Opin Biol Ther 2018 Jun;18(6):619-631. Epub 2018 May 8 doi: 10.1080/14712598.2018.1468885. PMID: 29681203
Diagnosis, treatment and prognosis of Guillain-Barré syndrome (GBS).
van Doorn PA
Presse Med 2013 Jun;42(6 Pt 2):e193-201. Epub 2013 Apr 28 doi: 10.1016/j.lpm.2013.02.328. PMID: 23628447
Guillain-Barré syndrome.
Hughes RA, Cornblath DR
Lancet 2005 Nov 5;366(9497):1653-66. doi: 10.1016/S0140-6736(05)67665-9. PMID: 16271648
Guillain-Barré syndrome: epidemiology, pathophysiology and management.
Kuwabara S
Drugs 2004;64(6):597-610. doi: 10.2165/00003495-200464060-00003. PMID: 15018590

Clinical prediction guides

Clinical and Electrophysiological Features of Chronic Motor Axonal Neuropathy.
Zhang D, Geng H, Cao L, Li W
J Clin Neurophysiol 2022 May 1;39(4):317-323. Epub 2020 Nov 20 doi: 10.1097/WNP.0000000000000771. PMID: 32852287
Epidemiological and clinical aspects of Guillain-Barré syndrome and its variants.
Oliveira DRDCAB, Fernandez RNM, Grippe TC, Baião FS, Duarte RL, Fernandez DJ
Arq Neuropsiquiatr 2021 Jun;79(6):497-503. doi: 10.1590/0004-282X-ANP-2020-0314. PMID: 34320055Free PMC Article
The immunotherapy of Guillain-Barré syndrome.
Restrepo-Jiménez P, Rodríguez Y, González P, Chang C, Gershwin ME, Anaya JM
Expert Opin Biol Ther 2018 Jun;18(6):619-631. Epub 2018 May 8 doi: 10.1080/14712598.2018.1468885. PMID: 29681203
Guillain-Barré syndrome in Asia.
Bae JS, Yuki N, Kuwabara S, Kim JK, Vucic S, Lin CS, Kiernan MC
J Neurol Neurosurg Psychiatry 2014 Aug;85(8):907-13. Epub 2013 Dec 19 doi: 10.1136/jnnp-2013-306212. PMID: 24357682
Diagnosis, treatment and prognosis of Guillain-Barré syndrome (GBS).
van Doorn PA
Presse Med 2013 Jun;42(6 Pt 2):e193-201. Epub 2013 Apr 28 doi: 10.1016/j.lpm.2013.02.328. PMID: 23628447

Recent systematic reviews

Triggers of Guillain-Barré Syndrome: Campylobacter jejuni Predominates.
Finsterer J
Int J Mol Sci 2022 Nov 17;23(22) doi: 10.3390/ijms232214222. PMID: 36430700Free PMC Article
Systematic review of cases of acute myelitis in individuals with COVID-19.
Schulte EC, Hauer L, Kunz AB, Sellner J
Eur J Neurol 2021 Oct;28(10):3230-3244. Epub 2021 Jul 12 doi: 10.1111/ene.14952. PMID: 34060708Free PMC Article
Hyper-reflexia in Guillain-Barré syndrome: systematic review.
Uncini A, Notturno F, Kuwabara S
J Neurol Neurosurg Psychiatry 2020 Mar;91(3):278-284. Epub 2020 Jan 14 doi: 10.1136/jnnp-2019-321890. PMID: 31937584
Predictors of respiratory failure in patients with Guillain-Barré syndrome: a systematic review and meta-analysis.
Green C, Baker T, Subramaniam A
Med J Aust 2018 Mar 5;208(4):181-188. doi: 10.5694/mja17.00552. PMID: 29490222
Associations between tumor necrosis factor-α gene polymorphisms and the risk of Guillain-Barré syndrome and its subtypes: A systematic review and meta-analysis.
Liu J, Lian Z, Chen H, Shi Z, Feng H, Du Q, Zhang Q, Zhou H
J Neuroimmunol 2017 Dec 15;313:25-33. Epub 2017 Oct 7 doi: 10.1016/j.jneuroim.2017.10.003. PMID: 29153605

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