Guillain-Barré syndrome pathophysiology: Difference between revisions
Jump to navigation
Jump to search
No edit summary |
No edit summary |
||
| Line 31: | Line 31: | ||
* Campylobacter jejuni LPS contains antigens resembling GM1 and GQ1b. | * 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.<ref name="pmid8228822">{{cite journal |vauthors=Yuki N, Taki T, Inagaki F, Kasama T, Takahashi M, Saito K, Handa S, Miyatake T |title=A bacterium lipopolysaccharide that elicits Guillain-Barré syndrome has a GM1 ganglioside-like structure |journal=J. Exp. Med. |volume=178 |issue=5 |pages=1771–5 |date=November 1993 |pmid=8228822 |pmc=2191246 |doi= |url=}}</ref><ref name="pmid8413947">{{cite journal |vauthors=Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I |title=Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies |journal=Neurology |volume=43 |issue=10 |pages=1911–7 |date=October 1993 |pmid=8413947 |doi= |url=}}</ref> | * 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.<ref name="pmid8228822">{{cite journal |vauthors=Yuki N, Taki T, Inagaki F, Kasama T, Takahashi M, Saito K, Handa S, Miyatake T |title=A bacterium lipopolysaccharide that elicits Guillain-Barré syndrome has a GM1 ganglioside-like structure |journal=J. Exp. Med. |volume=178 |issue=5 |pages=1771–5 |date=November 1993 |pmid=8228822 |pmc=2191246 |doi= |url=}}</ref><ref name="pmid8413947">{{cite journal |vauthors=Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I |title=Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies |journal=Neurology |volume=43 |issue=10 |pages=1911–7 |date=October 1993 |pmid=8413947 |doi= |url=}}</ref> | ||
* In the serum of GBS patients with CMV as the antecedent infection, we may see antibodies against GM2. | * In the serum of GBS patients with CMV as the antecedent infection, we may see antibodies against GM2.<ref name="pmid8784256">{{cite journal |vauthors=Irie S, Saito T, Nakamura K, Kanazawa N, Ogino M, Nukazawa T, Ito H, Tamai Y, Kowa H |title=Association of anti-GM2 antibodies in Guillain-Barré syndrome with acute cytomegalovirus infection |journal=J. Neuroimmunol. |volume=68 |issue=1-2 |pages=19–26 |date=August 1996 |pmid=8784256 |doi= |url=}}</ref> | ||
* 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. | * 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.<ref name="pmid10214761">{{cite journal |vauthors=Mori M, Kuwabara S, Miyake M, Dezawa M, Adachi-Usami E, Kuroki H, Noda M, Hattori T |title=Haemophilus influenzae has a GM1 ganglioside-like structure and elicits Guillain-Barré syndrome |journal=Neurology |volume=52 |issue=6 |pages=1282–4 |date=April 1999 |pmid=10214761 |doi= |url=}}</ref> | ||
* 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. | * 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: | * there are 4 types of GBS with different pathological sequences of events: | ||
Revision as of 18:54, 20 December 2018
|
Guillain-Barré syndrome Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Guillain-Barré syndrome pathophysiology On the Web |
|
American Roentgen Ray Society Images of Guillain-Barré syndrome pathophysiology |
|
Risk calculators and risk factors for Guillain-Barré syndrome pathophysiology |
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:
- 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:
- 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:
- 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:
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.[9]
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.
- ↑ 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.