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Acute motor and sensory axonal neuropathy

MedGen UID:
859705
•Concept ID:
C3900111
•
Disease or Syndrome
Synonyms: Acute Motor and Sensory Axonal Neuropathy; acute motor and sensory axonal neuropathy; Acute motor sensory axonal Guillain-Barré syndrome; Acute motor sensory axonal neuropathy; Acute motor-sensory axonal GBS; acute motor-sensory axonal GBS; acute motor-sensory axonal Guillain-BarrC) syndrome; Acute motor-sensory axonal Guillain-Barré syndrome; acute motor-sensory axonal Guillain-Barré syndrome; Acute Motor-Sensory Axonal Neuropathy; acute motor-sensory axonal neuropathy; AMSAN
SNOMED CT: Acute motor sensory axonal neuropathy (716722005); Acute motor sensory axonal Guillain-Barré syndrome (716722005)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on a mixture of major and minor genetic determinants possibly together with environmental factors. Diseases inherited in this manner are termed complex diseases.
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:0020348
Orphanet: ORPHA98917

Definition

A motor sensory axonal form of Guillain-Barré syndrome. Patients present with muscle weakness and sensory deficits, similar to that of the more frequent demyelinating form of Guillain-Barré syndrome. As in other types of Guillain-Barré syndrome, an infectious disease precedes the onset of limb weakness in the majority of cases. Although the exact pathological mechanism is poorly understood the disease is associated with the presence of antiganglioside antibodies and may be caused by antibody-mediated primary axonal degeneration or antibody-mediated inhibition of voltage-gated sodium channels. [from SNOMEDCT_US]

Term Hierarchy

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

Professional guidelines

PubMed

Electrodiagnostic criteria for Guillain-Barrè syndrome: a critical revision and the need for an update.
Uncini A, Kuwabara S
Clin Neurophysiol 2012 Aug;123(8):1487-95. Epub 2012 Apr 4 doi: 10.1016/j.clinph.2012.01.025. PMID: 22480600
Treatment of acute immune-mediated neuropathies: Guillain-Barré syndrome and clinical variants.
Sederholm BH
Semin Neurol 2010 Sep;30(4):365-72. Epub 2010 Oct 12 doi: 10.1055/s-0030-1267280. PMID: 20941669
Treatment of immune-mediated, dysimmune neuropathies.
Finsterer J
Acta Neurol Scand 2005 Aug;112(2):115-25. doi: 10.1111/j.1600-0404.2005.00448.x. PMID: 16008538

Recent clinical studies

Etiology

Clinical features and real-world outcomes of Guillain-Barré syndrome in the Philippines.
I Espiritu A, Separa KJNJ, Milla FJC, Adiao KJB, Leochico CFD, Jamora RDG
Neurol Res 2021 Dec;43(12):995-1004. Epub 2021 Jul 6 doi: 10.1080/01616412.2021.1948750. PMID: 34229572
Clinical, electrophysiological findings and evaluation of prognosis of patients with Guillain-Barré syndrome.
Kılıç B, Güngör S, Özgör B
Turk J Pediatr 2019;61(2):200-208. doi: 10.24953/turkjped.2019.02.008. PMID: 32077646
Clinical and electrophysiological characteristics of Guillain-Barré syndrome in Colombia.
Gómez Á, Díaz A, Carrión-Penagos J, Reyes J, Reyes S
J Peripher Nerv Syst 2019 Sep;24(3):268-271. Epub 2019 Aug 18 doi: 10.1111/jns.12340. PMID: 31386254
Sonographic evaluation of peripheral nerves in subtypes of Guillain-Barré syndrome.
Mori A, Nodera H, Takamatsu N, Maruyama-Saladini K, Osaki Y, Shimatani Y, Kaji R
J Neurol Sci 2016 May 15;364:154-9. Epub 2016 Mar 26 doi: 10.1016/j.jns.2016.03.042. PMID: 27084237
Guillain-Barré syndrome in the elderly.
Peric S, Berisavac I, Stojiljkovic Tamas O, Rajic S, Babic M, Cvijanovic M, Dominovic-Kovacevic A, Basta I, Beslac-Bumbasirevic L, Lavrnic D
J Peripher Nerv Syst 2016 Jun;21(2):105-10. doi: 10.1111/jns.12163. PMID: 26880714

Diagnosis

The value of sensory nerve conduction studies in the diagnosis of Guillain-Barré syndrome.
Freiha J, Zoghaib R, Makhoul K, Maalouf N, Riachi N, Chalah MA, Ayache SS, Ahdab R
Clin Neurophysiol 2021 May;132(5):1157-1162. Epub 2021 Mar 13 doi: 10.1016/j.clinph.2021.02.013. PMID: 33780722
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
The Guillain-Barré syndrome.
Fujimura H
Handb Clin Neurol 2013;115:383-402. doi: 10.1016/B978-0-444-52902-2.00021-7. PMID: 23931791
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
Natura non facit saltus in anti-ganglioside antibody-mediated neuropathies.
Uncini A, Notturno F, Capasso M
Muscle Nerve 2013 Oct;48(4):484-7. doi: 10.1002/mus.23881. PMID: 23625341
Guillain-Barré syndrome: an update.
Vucic S, Kiernan MC, Cornblath DR
J Clin Neurosci 2009 Jun;16(6):733-41. Epub 2009 Apr 7 doi: 10.1016/j.jocn.2008.08.033. PMID: 19356935
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
Acquired inflammatory demyelinating neuropathies.
Ensrud ER, Krivickas LS
Phys Med Rehabil Clin N Am 2001 May;12(2):321-34, ix. PMID: 11345010

Prognosis

The value of sensory nerve conduction studies in the diagnosis of Guillain-Barré syndrome.
Freiha J, Zoghaib R, Makhoul K, Maalouf N, Riachi N, Chalah MA, Ayache SS, Ahdab R
Clin Neurophysiol 2021 May;132(5):1157-1162. Epub 2021 Mar 13 doi: 10.1016/j.clinph.2021.02.013. PMID: 33780722
Concurrent acute motor and sensory axonal neuropathy and immune thrombocytopenic purpura.
Ward IM, Fewell AE, Ferraro DM, Morris MJ
Mil Med 2013 Mar;178(3):e367-71. doi: 10.7205/MILMED-D-12-00306. PMID: 23707127
Electrodiagnostic criteria for Guillain-Barrè syndrome: a critical revision and the need for an update.
Uncini A, Kuwabara S
Clin Neurophysiol 2012 Aug;123(8):1487-95. Epub 2012 Apr 4 doi: 10.1016/j.clinph.2012.01.025. PMID: 22480600
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
Acquired inflammatory demyelinating neuropathies.
Ensrud ER, Krivickas LS
Phys Med Rehabil Clin N Am 2001 May;12(2):321-34, ix. PMID: 11345010

Clinical prediction guides

Clinical features and real-world outcomes of Guillain-Barré syndrome in the Philippines.
I Espiritu A, Separa KJNJ, Milla FJC, Adiao KJB, Leochico CFD, Jamora RDG
Neurol Res 2021 Dec;43(12):995-1004. Epub 2021 Jul 6 doi: 10.1080/01616412.2021.1948750. PMID: 34229572
The value of sensory nerve conduction studies in the diagnosis of Guillain-Barré syndrome.
Freiha J, Zoghaib R, Makhoul K, Maalouf N, Riachi N, Chalah MA, Ayache SS, Ahdab R
Clin Neurophysiol 2021 May;132(5):1157-1162. Epub 2021 Mar 13 doi: 10.1016/j.clinph.2021.02.013. PMID: 33780722
Clinical, electrophysiological findings and evaluation of prognosis of patients with Guillain-Barré syndrome.
Kılıç B, Güngör S, Özgör B
Turk J Pediatr 2019;61(2):200-208. doi: 10.24953/turkjped.2019.02.008. PMID: 32077646
Guillain-Barré syndrome in the elderly.
Peric S, Berisavac I, Stojiljkovic Tamas O, Rajic S, Babic M, Cvijanovic M, Dominovic-Kovacevic A, Basta I, Beslac-Bumbasirevic L, Lavrnic D
J Peripher Nerv Syst 2016 Jun;21(2):105-10. doi: 10.1111/jns.12163. PMID: 26880714
Transverse myelitis and acute motor sensory axonal neuropathy due to Legionella pneumophila: a case report.
Canpolat M, Kumandas S, Yikilmaz A, Gumus H, Koseoglu E, Poyrazoğlu HG, Kose M, Per H
Pediatr Int 2013 Dec;55(6):778-82. doi: 10.1111/ped.12126. PMID: 24330286

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