Guillain-Barré syndrome pathophysiology: Difference between revisions
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*** Complement components will deposit in the outer surface of schwann cell membrane and starts the process of myelin disruption. | *** Complement components will deposit in the outer surface of schwann cell membrane and starts the process of myelin disruption. | ||
*** Macrophages will infiltrate and complete the demyelination. | *** Macrophages will infiltrate and complete the demyelination. | ||
** AMAN:<ref name="pmid8871584">{{cite journal |vauthors=Hafer-Macko C, Hsieh ST, Li CY, Ho TW, Sheikh K, Cornblath DR, McKhann GM, Asbury AK, Griffin JW |title=Acute motor axonal neuropathy: an antibody-mediated attack on axolemma |journal=Ann. Neurol. |volume=40 |issue=4 |pages=635–44 |date=October 1996 |pmid=8871584 |doi=10.1002/ana.410400414 |url=}}</ref> | ** AMAN:<ref name="pmid8871584">{{cite journal |vauthors=Hafer-Macko C, Hsieh ST, Li CY, Ho TW, Sheikh K, Cornblath DR, McKhann GM, Asbury AK, Griffin JW |title=Acute motor axonal neuropathy: an antibody-mediated attack on axolemma |journal=Ann. Neurol. |volume=40 |issue=4 |pages=635–44 |date=October 1996 |pmid=8871584 |doi=10.1002/ana.410400414 |url=}}</ref><ref name="pmid6502752">{{cite journal |vauthors=Ganser AL, Kirschner DA |title=Differential expression of gangliosides on the surfaces of myelinated nerve fibers |journal=J. Neurosci. Res. |volume=12 |issue=2-3 |pages=245–55 |date=1984 |pmid=6502752 |doi=10.1002/jnr.490120212 |url=}}</ref> | ||
*** IgG and activated complement attack to the axolemma of motor fibers in ranvier nodes. | *** IgG and activated complement attack to the axolemma of motor fibers in ranvier nodes. | ||
*** Macrophages will migrate to these nodes and separate the axon from overlying shcwann cells and destroy the axolemma. | *** Macrophages will migrate to these nodes and separate the axon from overlying shcwann cells and destroy the axolemma. | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D.
Overview
Pathophysiology
Physiology
- Soma is the neuronal cell body which is a closed area with cell membrane.
- Dendrites are branched processes which lead the impulse into the neuronal cell body.
- Axons in a single process which lead the impulse away from the neuronal cell body.
- Myelin sheath is the oligodendrocyte membrane which wraps around the axons.
- Myelin sheath is insulated against electrical impulses and is separated by nodes of ranvier which can transfer the electrical impulse.
- This structure leads to fast traveling of electrical impulses.[1]
Pathogenesis
- The exact pathogenesis of Guillain Barre syndrome is not completely understood but in 2/3 of cases there is a history of an infectious disease in the past month.[2]
- The most common pathogens responsible for these antecedent infections are:[3][4]
- Campylobacter jejuni
- Cytpmegalo virus
- Hemphilus influanza
- It is believed that the main underlying etiology of GBS is an autoimmune reaction.
- The main theory explaining the relation between these infections and GBS is molecular mimicry.
- There are many antigens on the surface of these pathogens which are similar to myelin sheath or axonal proteins.
- Campylobacter jejuni LPS contains antigens resembling GM1 and GQ1b.
- In the serum of GBS patients with campylobacter jejuni as the antecedent infection, we may see antibodies against GM1 and GQ1b which can cause AMAN and Miller Fisher respectively.[5][6]
- In the serum of GBS patients with CMV as the antecedent infection, we may see antibodies against GM2.[7]
- Haemophilus influenzae have GM1 like structure on its surface and in the serum of patients with haemophilus influenzae related GBS we may see antibodies against GM1.[8]
- although antibody formation and humoral immunity said to be the underlying pathophysiology mechanism, sometimes pathologic findings underscore the importance of circulating antibodies in the pathogenesis of GBS.
- there are 4 types of GBS with different pathological sequences of events:
- AIDP:[9]
- Lymphocytes will infiltrate in peripheral nerves and nerves root of spinal cord.
- Complement components will deposit in the outer surface of schwann cell membrane and starts the process of myelin disruption.
- Macrophages will infiltrate and complete the demyelination.
- AMAN:[10][11]
- IgG and activated complement attack to the axolemma of motor fibers in ranvier nodes.
- Macrophages will migrate to these nodes and separate the axon from overlying shcwann cells and destroy the axolemma.
- AMSAN:[12][13]
- Very little lymphocyte infiltration
- direct attack to the axon of motor and sensory neurons.
- Miller Fisher:
- IgG antibody against GQ1b ganglioside which is present in the ocolomotor nerve, cerebellar neurons and dorsal root ganglion cells.
- It seems that antibody-mediated mechanism is more prominent in AMAN, whereas cellular mechanisms are more important in AIDP.
- AIDP:[9]
Genetics
- There is no characteristic genetic association with GBS.
Microscopic Pathology
- On microscopic histopathological analysis:
- AIDP: Lymphocyte and macrophage infiltration, demyelination.
- AMAN: Macrophage infiltration and axolemma disruption in motor fibers.
- AMSAN: Disruption of both motor and sensory fibers. Little lymphocyte infiltration.
- Miller Fisher: Oculomotor nerve demyelination.[14]
References
- ↑ Mattle, Heinrich (2017). Fundamentals of neurology : an illustrated guide. Stuttgart New York: Thieme. ISBN 9783131364524.
- ↑ Winer JB, Hughes RA, Anderson MJ, Jones DM, Kangro H, Watkins RP (May 1988). "A prospective study of acute idiopathic neuropathy. II. Antecedent events". J. Neurol. Neurosurg. Psychiatry. 51 (5): 613–8. PMC 1033063. PMID 3404161.
- ↑ Jacobs BC, Rothbarth PH, van der Meché FG, Herbrink P, Schmitz PI, de Klerk MA, van Doorn PA (October 1998). "The spectrum of antecedent infections in Guillain-Barré syndrome: a case-control study". Neurology. 51 (4): 1110–5. PMID 9781538.
- ↑ Ogawara K, Kuwabara S, Mori M, Hattori T, Koga M, Yuki N (October 2000). "Axonal Guillain-Barré syndrome: relation to anti-ganglioside antibodies and Campylobacter jejuni infection in Japan". Ann. Neurol. 48 (4): 624–31. PMID 11026446.
- ↑ Yuki N, Taki T, Inagaki F, Kasama T, Takahashi M, Saito K, Handa S, Miyatake T (November 1993). "A bacterium lipopolysaccharide that elicits Guillain-Barré syndrome has a GM1 ganglioside-like structure". J. Exp. Med. 178 (5): 1771–5. PMC 2191246. PMID 8228822.
- ↑ Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I (October 1993). "Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies". Neurology. 43 (10): 1911–7. PMID 8413947.
- ↑ Irie S, Saito T, Nakamura K, Kanazawa N, Ogino M, Nukazawa T, Ito H, Tamai Y, Kowa H (August 1996). "Association of anti-GM2 antibodies in Guillain-Barré syndrome with acute cytomegalovirus infection". J. Neuroimmunol. 68 (1–2): 19–26. PMID 8784256.
- ↑ Mori M, Kuwabara S, Miyake M, Dezawa M, Adachi-Usami E, Kuroki H, Noda M, Hattori T (April 1999). "Haemophilus influenzae has a GM1 ganglioside-like structure and elicits Guillain-Barré syndrome". Neurology. 52 (6): 1282–4. PMID 10214761.
- ↑ Hafer-Macko CE, Sheikh KA, Li CY, Ho TW, Cornblath DR, McKhann GM, Asbury AK, Griffin JW (May 1996). "Immune attack on the Schwann cell surface in acute inflammatory demyelinating polyneuropathy". Ann. Neurol. 39 (5): 625–35. doi:10.1002/ana.410390512. PMID 8619548.
- ↑ Hafer-Macko C, Hsieh ST, Li CY, Ho TW, Sheikh K, Cornblath DR, McKhann GM, Asbury AK, Griffin JW (October 1996). "Acute motor axonal neuropathy: an antibody-mediated attack on axolemma". Ann. Neurol. 40 (4): 635–44. doi:10.1002/ana.410400414. PMID 8871584.
- ↑ Ganser AL, Kirschner DA (1984). "Differential expression of gangliosides on the surfaces of myelinated nerve fibers". J. Neurosci. Res. 12 (2–3): 245–55. doi:10.1002/jnr.490120212. PMID 6502752.
- ↑ Feasby TE, Hahn AF, Brown WF, Bolton CF, Gilbert JJ, Koopman WJ (June 1993). "Severe axonal degeneration in acute Guillain-Barré syndrome: evidence of two different mechanisms?". J. Neurol. Sci. 116 (2): 185–92. PMID 8336165.
- ↑ Feasby TE, Gilbert JJ, Brown WF, Bolton CF, Hahn AF, Koopman WF, Zochodne DW (December 1986). "An acute axonal form of Guillain-Barré polyneuropathy". Brain. 109 ( Pt 6): 1115–26. PMID 3790970.
- ↑ Phillips MS, Stewart S, Anderson JR (May 1984). "Neuropathological findings in Miller Fisher syndrome". J. Neurol. Neurosurg. Psychiatry. 47 (5): 492–5. PMC 1027825. PMID 6736980.