Guillain-Barré syndrome: Difference between revisions

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== Background ==
==Background==


=== Types ===
* Causes either acute inflammatory demyelinating polyneuropathy, or acute motor axonal neuropathy, or acute motor and sensory axonal neuropathy

*Guillain-Barré syndrome
**Acute inflammatory demyelinating polyneuropathy (AIDP)
**Acute motor axonal neuropathy
***Acute motor-sensory axonal neuropathy
***Acute motor-conduction-block neuropathy
***Pharyngeal-cervical-branchial weakness
*Miller Fisher syndrome
**Incomplete MFS
***Acute motor-ophthalmoparesis, without ataxia
***Acute ataxic neyropathy, without ophthalmoparesis
**Bickerstaff's brainstem encephalitis


===Etiology===
===Etiology===


*''[[Campylobacter jejuni]]''
*''[[Campylobacter jejuni|'''Campylobacter jejuni''']]''
*[[Cytomegalovirus]]
*[[Cytomegalovirus]], [[Haemophilus influenzae]], [[Mycoplasma pneumoniae]], [[Influenza]], [[HIV]]
*Also [[COVID-19]], [[Zika virus]], [[EBV]], [[HSV]], [[VZV]], [[Chikungunya]], [[Escherichia coli]], [[Japanese encephalitis virus]]
*[[Epstein-Barr virus]]
*Non-infectious causes
*[[Zika virus]]
*[[HIV]]
**[[Immunization]]
**Surgery, trauma
*Non-infectious causes, including immunization, surgery, trauma, and hematopoietic stem cell transplantation
**Medications, including [[TNF-α inhibitors]], [[tacrolimus]], [[suramin]], [[isotretinoin]], and [[immune checkpoint inhibitors]]
**[[Hematopoietic stem cell transplantation]]

=== Pathophysiology ===

* Immune crossreactivity causes autoimmune destruction of either the myelin sheath (AIDP) or the axon itself
* Most common ADAM antibodies are anti-GM1 or anti-GD1a IgG, which recognize gangliosides
* Most common MFS antibody is anti-GQ1b IgG (in 90%)

⚫
===Epidemiology===

⚫
*About 1 case per 100,000 people per year in Western countries

==Clinical Manifestations==

⚫
*Ascending paralysis ± sensory involvement
⚫
*Usually occurs 1 to 2 weeks after an immune-stimulating even such as illness or vaccination
⚫
**Preceding illness usually a gastrointestinal or respiratory infection
⚫
**[[Campylobacter jejuni]] is the most common causative organism identified
⚫
**Rare following influenza vaccination; most notable was the 1976 H1N1 vaccine, with about 1 in 100,000 vaccine recipients developing GBS


== Differential Diagnosis ==
⚫
=== Epidemiology ===


* [[CIDP]]
⚫
* About 1 case per 100,000 people per year in Western countries
* [[Thiamine deficiency]]
* Polyneuropathy, including [[Arsenic poisoning|acute arsenic poisoning]] and other toxin-induced peripheral polyneuropathy, [[Lyme disease]], [[tick paralysis]], [[vasculitis]], [[sarcoidosis]], [[porphyria]]
* [[Leptomeningeal lymphoma]]
* [[Paraneoplastic syndrome]]
* [[Spinal cord disorder]]
* [[Botulism]], [[myasthenia gravis]], [[Lambert-Eaton syndrome]]
* Acute [[polymyositis]], [[dermatomyositis]], [[necrotizing myopathy]], [[critical illness neuropathy]]


== Clinical Manifestations ==
== Investigations ==


* Lumbar puncture, with CSF classically showing albuminocytologic dissociation (elevated protein with white count less than 50), though neither normal protein levels nor elevated white cells exclude the diagnosis[[CiteRef::al-hakem2023cs]]
⚫
* Ascending paralysis ± sensory involvement
⚫
* Usually occurs 1 to 2 weeks after an immune-stimulating even such as illness or vaccination
⚫
** Preceding illness usually a gastrointestinal or respiratory infection
⚫
** [[Campylobacter jejuni]] is the most common causative organism identified
⚫
** Rare following influenza vaccination; most notable was the 1976 H1N1 vaccine, with about 1 in 100,000 vaccine recipients developing GBS


== Further Reading ==
==Further Reading==


* Guillain-Barré syndrome. ''Lancet''. 2016;388:717-727. doi: [https://doi.org/10.1016/S0140-6736(16)00339-1 10.1016/S0140-6736(16)00339-1]
*Guillain-Barré syndrome. ''Lancet''. 2016;388:717-727. doi: [https://doi.org/10.1016/S0140-6736(16)00339-1 10.1016/S0140-6736(16)00339-1]


[[Category:Neurology]]
[[Category:Neurology]]

Latest revision as of 17:56, 30 September 2024

Background

Types

  • Guillain-Barré syndrome
    • Acute inflammatory demyelinating polyneuropathy (AIDP)
    • Acute motor axonal neuropathy
      • Acute motor-sensory axonal neuropathy
      • Acute motor-conduction-block neuropathy
      • Pharyngeal-cervical-branchial weakness
  • Miller Fisher syndrome
    • Incomplete MFS
      • Acute motor-ophthalmoparesis, without ataxia
      • Acute ataxic neyropathy, without ophthalmoparesis
    • Bickerstaff's brainstem encephalitis

Etiology

Pathophysiology

  • Immune crossreactivity causes autoimmune destruction of either the myelin sheath (AIDP) or the axon itself
  • Most common ADAM antibodies are anti-GM1 or anti-GD1a IgG, which recognize gangliosides
  • Most common MFS antibody is anti-GQ1b IgG (in 90%)

Epidemiology

  • About 1 case per 100,000 people per year in Western countries

Clinical Manifestations

  • Ascending paralysis ± sensory involvement
  • Usually occurs 1 to 2 weeks after an immune-stimulating even such as illness or vaccination
    • Preceding illness usually a gastrointestinal or respiratory infection
    • Campylobacter jejuni is the most common causative organism identified
    • Rare following influenza vaccination; most notable was the 1976 H1N1 vaccine, with about 1 in 100,000 vaccine recipients developing GBS

Differential Diagnosis

Investigations

  • Lumbar puncture, with CSF classically showing albuminocytologic dissociation (elevated protein with white count less than 50), though neither normal protein levels nor elevated white cells exclude the diagnosis1

Further Reading

References

  1. ^  Helle Al-Hakem, Alex Y. Doets, Amro Maher Stino, Sasha A. Zivkovic, Henning Andersen, Hugh J. Willison, David R. Cornblath, Kenneth C. Gorson, Zhahirul Islam, Quazi Deen Mohammad, Søren Hein Sindrup, Susumu Kusunoki, Amy Davidson, Carlos Casasnovas, Kathleen Bateman, James A.L. Miller, Bianca van den Berg, Christine Verboon, Joyce Roodbol, Sonja E. Leonhard, Samuel Arends, Linda W.G. Luijten, Luana Benedetti, Satoshi Kuwabara, Peter Van den Bergh, Soledad Monges, Girolama A. Marfia, Nortina Shahrizaila, Giuliana Galassi, Yann Pereon, Jan Bürmann, Krista Kuitwaard, Ruud P. Kleyweg, Cintia Marchesoni, María J. Sedano Tous, Luis Querol, Lorena Martín-Aguilar, Yuzhong Wang, Eduardo Nobile-Orazio, Simon Rinaldi, Angelo Schenone, Julio Pardo, Frederique H. Vermeij, Waqar Waheed, Helmar C. Lehmann, Volkan Granit, Beth Stein, Guido Cavaletti, Gerardo Gutiérrez-Gutiérrez, Fabio A. Barroso, Leo H. Visser, Hans D. Katzberg, Efthimios Dardiotis, Shahram Attarian, Anneke J. van der Kooi, Filip Eftimov, Paul W. Wirtz, Johnny P.A. Samijn, H. Jacobus Gilhuis, Robert D.M. Hadden, James K.L. Holt, Kazim A. Sheikh, Noah Kolb, Summer Karafiath, Michal Vytopil, Giovanni Antonini, Thomas E. Feasby, Catharina Faber, Hans Kramers, Mark Busby, Rhys C. Roberts, Nicholas J. Silvestri, Raffaella Fazio, Gert W. van Dijk, Marcel P.J. Garssen, Jan Verschuuren, Thomas Harbo, Bart C. Jacobs, R.A.C. Hughes, H.P. Hartung, L.C. de Koning, M. Mandarakas, M. van Woerkom, R.C. Reisin, S.W. Reddel, S.T. Hsieh, J.M. Addington, S. Ajroud-Driss, L. Alessandro, U.A. Badrising, G. Balloy, I.R. Bella, T.E. Bertorini, R. Bhavaraju-Sanka, M. Bianco, T.H. Brannagan, K. Brennan, C. Briani, S. Butterworth, C.C. Chao, S. Chen, K.G. Claeys, M.E. Conti, J.S. Cosgrove, M.C. Dalakas, M. Derejko, M.M. Dimachkie, A. Echaniz-Laguna, C. Fokke, T. Fujioka, E.A. Fulgenzi, T. García Sobrino, C.J. Gijsbers, J.M. Gilchrist, J.M. Goldstein, J. Goodfellow, N.A. Goyal, S. Grisanti, L. Gutmann, M. Htut, K. Jellema, I. Jericó Pascual, M.C. Jimeno-Montero, K. Kaida, H. Kerkhoff, M. Khoshnoodi, L. Kiers, M. Kuwahara, J.Y. Kwan, S.S. Ladha, L. Landschoff Lassen, V. Lawson, E. Lee Pan, M.P.T. Lunn, H. Manji, C. Márquez Infante, E. Martinez Hernandez, G. Mataluni, M.G. Mattiazzi, C.J. McDermott, G.D. Meekins, G. Morís de la Tassa, C. Nascimbene, R.J. Nowak, P. Orizaola, M. Osei-Bonsu, A. Pardal, E. Pascual-Goñi, R.M. Pascuzzi, V. Prada, M. Pulley, I. Rojas-Marcos, S.A. Rudnicki, G.M. Sachs, L. Santoro, A. Savransky, L. Schwindling, Y. Sekiguchi, C.L. Sommer, C-Y Tan, H. Tankisi, P.T. Twydell, P. van Damme, T. van der Ree, R. van Koningsveld, J.D. Varrato, V. Vélez Santamaria, C. Walgaard, Y. Yamagishi, L. Zhang, MM, L. Zhou. CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome. Neurology. 2023;100(23). doi:10.1212/wnl.0000000000207282.

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