Materiały źródłowe

• #1 Guillain–Barré syndrome: pathogenesis, diagnosis, treatment and prognosis | Nature Reviews Neurology

  https://www.nature.com/articles/nrneurol.2014.121

  GuillainBarr syndrome (GBS) is a heterogeneous disease characterized by rapidly progressive, symmetrical limb weakness with hyporeflexia or areflexia; sensory disturbances and cranial nerve deficits occur in some patients. […] Molecular mimicry, antiganglioside antibodies and, likely, complement activation are involved in the pathogenesis of GBS; a potential role for genetic susceptibility requires further investigation. […] Molecular mimicry of pathogen-borne antigens, leading to generation of crossreactive antibodies that also target gangliosides, is part of the pathogenesis of GBS; the subtype and severity of the syndrome are partly determined by the nature of the antecedent infection and specificity of such antibodies. […] The immunobiology of GuillainBarr syndromes. […] Pathophysiological actions of neuropathy-related anti-ganglioside antibodies at the neuromuscular junction.

• #2 Toll-like receptor 2 and -4 are involved in the pathogenesis of the Guillain-Barré syndrome

  https://www.spandidos-publications.com/10.3892/mmr.2015.3730

  Guillain-Barr syndrome (GBS) is an autoimmune disorder of the peripheral nervous system characterized by weakness in the limbs. To date, numerous hypotheses have been suggested to explain the pathogenesis of GBS; however, the pathogenesis of GBS remains to be elucidated. […] The aim of the present study was to investigate the association between Toll-like receptor (TLR) 2, TLR4 and GBS. […] The results revealed that the mRNA levels of TLR2, TLR4, MyD88 and NFB were significantly increased in patients with GBS compared with those in healthy controls (P=0.003, 0.017, 0.032 and 0.015, respectively). […] The results of the present study demonstrated that TLR2 and TLR4 are involved in the pathogenesis of GBS and that they and their associated signaling pathways may be targets for the treatment of GBS.

• #3

  https://www.who.int/news-room/fact-sheets/detail/guillain-barr%C3%A9-syndrome

  In Guillain-Barr syndrome, the body’s immune system attacks part of the peripheral nervous system. […] The cause of it is not fully understood, but most cases follow an infection with a virus or bacteria. This leads the immune system to attack the body itself. […] Given the autoimmune nature of the disease, its acute phase is typically treated with immunotherapy, such as plasma exchange to remove antibodies from the blood or intravenous immunoglobulin.

• #4 Guillain-Barre Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532254/

  Guillain-Barr syndrome (GBS) is a rare but serious post-infectious immune-mediated neuropathy. It results from the autoimmune destruction of nerves in the peripheral nervous system causing symptoms such as numbness, tingling, and weakness that can progress to paralysis. […] Guillain-Barr syndrome (GBS) and its variants are considered post-infectious, immune-mediated neuropathies. Evidence from animal models suggests a key role of molecular mimicry. In Campylobacter jejuni gastrointestinal infections, a lipooligosaccharide present in the outer membrane of the bacteria is similar to gangliosides which are components of the peripheral nerves. Therefore, an immune response triggered to fight infection can lead to a cross-reaction on host nerves. […] Antecedent infections are reported in up to 70% of patients with Guillain-Barr syndrome (GBS). Therefore, molecular mimicry plays a substantial role in our understanding of GBS, particularly the axonal variant. The lipooligosaccharide of Campylobacter jejuni is similar to the gangliosides of peripheral nerve membranes. Passive immunization of rabbits with these ganglioside-like lipooligosaccharides has led to similar clinical syndromes of flaccid tetraplegia, similar to the acute motor axonal neuropathy variant of GBS. Ganglioside antibodies have been shown to have different peripheral nerve targets. Anti-GD1a antibodies bind to paranodal myelin, nodes of Ranvier, and neuromuscular junction. GM1 and GQ1B antibodies bind to a peripheral nerve or neuromuscular junction. These different peripheral nerve targets may play a role in the heterogeneity of the clinical presentation of GBS. Additionally, the complement cascade is activated and plays a key role in the diseases pathogenesis. […] Less is known about the pathophysiology behind the acute inflammatory demyelinating polyneuropathy variant (AIDP) of GBS, although it is considered the most common variant in the United States.

• #5 Guillain-Barre Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/315632-overview

  GBS is a postinfectious, immune-mediated disease. Cellular and humoral immune mechanisms probably play a role in its development. Most patients report an infectious illness in the weeks prior to the onset of GBS. Many of the identified infectious agents are thought to induce production of antibodies that cross-react with specific gangliosides and glycolipids, such as GM1 and GD1b, that are distributed throughout the myelin in the peripheral nervous system. […] The pathophysiologic mechanism of an antecedent illness and of GBS can be typified by Campylobacter jejuni infections. […] Immune responses directed against lipopolysaccharide antigens in the capsule of C jejuni result in antibodies that cross-react with ganglioside GM1 in myelin, resulting in immunologic damage to the peripheral nervous system. This process has been termed molecular mimicry.

• #6 Guillain–Barré syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Guillain%E2%80%93Barr%C3%A9_syndrome

  GuillainBarr syndrome (GBS) is a rapid-onset muscle weakness caused by the immune system damaging the peripheral nervous system. […] Although the cause is unknown, the underlying mechanism involves an autoimmune disorder in which the body’s immune system mistakenly attacks the peripheral nerves and damages their myelin insulation. […] Different types of GuillainBarr syndrome feature different types of immune attacks. The demyelinating variant (AIDP) features damage to the myelin sheath by white blood cells (T lymphocytes and macrophages); this process is preceded by activation of a group of blood proteins known as complement. In contrast, the axonal variant is mediated by IgG antibodies and complement against the cell membrane covering the axon without direct lymphocyte involvement. […] Various antibodies directed at nerve cells have been reported in GuillainBarr syndrome. In the axonal subtype, these antibodies have been shown to bind to gangliosides, a group of substances found in peripheral nerves.

• #7 Guillain-Barré Syndrome | AAFP

  https://www.aafp.org/pubs/afp/issues/2013/0201/p191.html

  Guillain-Barr syndrome is believed to result from an aberrant immune response that attacks nerve tissue. […] The mechanism of GBS is believed to be an inflammatory neuropathy due to cross reactivity between neural antigens and antibodies that is induced by specific infections. Infectious organisms, such as C. jejuni, express lipooligosaccharides in the bacterial wall similar to gangliosides. This molecular mimicry creates antiganglioside antibodies that attack nerves. […] GBS has been shown to cause symptoms through multifocal areas of mononuclear cell infiltration in the peripheral nerves. The location and severity of the inflammation correspond to the clinical manifestations. In AIDP, the myelin is predominantly damaged, whereas in acute motor axonal neuropathy, the nodes of Ranvier are targeted.

• #8 Davidson College: Immune Condition: Guillain-Barre Syndrome

  https://www.bio.davidson.edu/movies/Immunology/Students/spring2006/Blumer/GBS.html

  Guillain-Barre Syndrome (GBS) is a rare, post-infectious, inflammatory autoimmune disease that is characterized by an ascending limb weakness and numbness in the extremities that can progress, in some cases, to paralysis (Yuki, 2005). […] The mechanism proposed thus far for the development of GBS is molecular mimicry. In summary, the body mounts an antibody response against a pathogen protein that resembles neural glycolipids (gangliosides), resulting in an attack on the peripheral nervous system. […] In the case of C. jejuni infection, antibodies will be produced, leading to activation of the complement system and phagocytosis of the bacteria. However, in rare cases the antibodies produced against certain C. jejuni antigens will also bind to gangliosides of the nervous tissue causing complement activation and damage by phagocytes. This results in damage to peripheral nervous tissues, which leads to demyelination and axonal damage (Kuwabara, 2004).

• #9 Autoimmune mechanisms in Guillain-Barré syndrome subtypes: a systematic review | Bulletin of Faculty of Physical Therapy | Full Text

  https://bfpt.springeropen.com/articles/10.1186/s43161-024-00258-8

  Guillain-Barr syndrome (GBS) is a complex autoimmune disorder characterized by acute onset of motor weakness, often following an infectious illness. The pathophysiology of GBS involves a multifaceted interplay between immune mechanisms and environmental factors, leading to demyelination or axonal degeneration. […] Key findings indicate that specific autoantibodies, such as anti-GM1 and anti-GQ1b, are associated with distinct subtypes of GBS, contributing to the diseases heterogeneity. Understanding these autoimmune mechanisms is crucial for improving diagnostic accuracy, therapeutic strategies, and prognostic indicators in GBS. […] Recent research emphasizes the importance of elucidating the distinct autoimmune mechanisms in each GBS subtype. This study seeks to provide evidence on specific autoimmune pathways that contribute to GBS and their clinical implications. It aims to identify unique autoimmune mechanisms within each GBS subtype, investigate how specific autoantibodies, such as anti-GM1 and anti-GQ1b, along with molecular mimicry, contribute to pathogenesis, and examine the correlation between clinical manifestations and autoimmune processes.

• #10 Autoimmune mechanisms in Guillain-Barré syndrome subtypes: a systematic review | Bulletin of Faculty of Physical Therapy | Full Text

  https://bfpt.springeropen.com/articles/10.1186/s43161-024-00258-8

  The autoimmune mechanisms underlying the major GBS subtypes vary significantly, as summarized in Table 5 below. Each subtype is associated with distinct immunopathological processes, which contribute to the clinical manifestations of the disease. AIDP is characterized by demyelination driven by T-cell activation and cytokine elevation, while AMAN and AMSAN are associated with anti-ganglioside antibodies leading to axonal degeneration. MFS involves molecular mimicry mechanisms leading to cranial nerve involvement. […] The predominant mechanism in AIDP is demyelination due to T-cell activation and elevated cytokines like TNF- and IL-6, which drive inflammatory responses leading to the destruction of myelin sheaths. […] Anti-GM1 antibodies are a key player in AMAN, where they target gangliosides on axonal membranes, causing axonal degeneration.

• #11 Autoimmune mechanisms in Guillain-Barré syndrome subtypes: a systematic review | Bulletin of Faculty of Physical Therapy | Full Text

  https://bfpt.springeropen.com/articles/10.1186/s43161-024-00258-8

  Anti-GM1 antibodies also feature prominently in AMSAN, but here the immune response involves more severe axonal damage, compounded by complement activation, which exacerbates nerve injury. […] Anti-GQ1b antibodies are the defining marker for MFS, causing cranial nerve dysfunction and leading to ophthalmoplegia and ataxia. […] The autoimmune mechanisms identified in this review have important clinical implications, particularly for diagnosis, treatment, and prognosis. […] High levels of anti-GM1 antibodies in AMAN and AMSAN are associated with more severe axonal damage and poorer recovery. […] Infections like COVID-19 and the Zika virus have been linked to the development of GBS through molecular mimicry, where viral antigens cross-react with peripheral nerves. […] Current therapeutic strategies for GBS, such as IVIG and plasmapheresis, are widely applied across all subtypes despite notable variations in effectiveness and safety among different subtypes.

• #12

  https://www.ijcmph.com/index.php/ijcmph/article/view/8242

  GuillainBarr syndrome (GBS) is a polyradiculoneuropathy autoimmune disease that is characterized by significant inflammation that affects the peripheral nervous system in a rapidly progressive pattern that is mainly clinically presented by muscle weakness. […] Among the reported antibodies, anti-GM1 and anti-GQ1B have been reported to be responsible for attacking and damaging either the neuromuscular junctions or peripheral nerves. […] Moreover, it has been found that the anti-GD1a antibodies in patients bind to the neuromuscular junction and also bind to the nodes of Ranvier of the peripheral nerves and the paranodal myelin of the affected nerves. […] Reports have shown that this disease is identified as special forms of neuropathies that develop in immune-mediated, post-infection sequelae.

• #13 Guillain–Barré syndrome: a century of progress | Nature Reviews Neurology

  https://www.nature.com/articles/nrneurol.2016.172

  In 1916, Guillain, Barr and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts novel findings that identified the disease we now know as GuillainBarr syndrome (GBS). […] Early clinicopathological and animal studies indicated that GBS was an immune-mediated demyelinating disorder, and that severe GBS could result in secondary axonal injury; the current treatments of plasma exchange and intravenous immunoglobulin, which were developed in the 1980s, are based on this premise. […] The association of Campylobacter jejuni strains has led to confirmation that anti-ganglioside antibodies are pathogenic and that axonal GBS involves an antibody and complement-mediated disruption of nodes of Ranvier, neuromuscular junctions and other neuronal and glial membranes. […] Now, ongoing clinical trials of the complement inhibitor eculizumab are the first targeted immunotherapy in GBS.

• #14 Guillain–Barre syndrome: demographics, clinical features, and outcome in a single tertiary care hospital, Oman | Neurosciences Journal

  https://nsj.org.sa/content/25/5/369

  The GBS is believed to include a variety of acute neuropathies with underlying immune-mediated pathogenic mechanisms rather than a single disease; therefore, GBS recognized variants are considered as syndromes [including acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor-sensory axonal neuropathy (AMSAN), and MillerFisher syndrome]. […] The most common variant of GBS among the studied patients was AIDP followed by AMAN. […] The seasonal and monthly variation may be attributed to sudden temperature differences in seasonal conditions, which makes certain months more prone to infections. […] The understanding of clinical presentation and epidemiology of GBS in each population can help us better understand the pathogenesis of the disease, its risk factors, and prognosis.

• #15 Guillain-Barre Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/315632-overview

  Pathologic findings in GBS include lymphocytic infiltration of spinal roots and peripheral nerves (cranial nerves may be involved as well), followed by macrophage-mediated, multifocal stripping of myelin. This phenomenon results in defects in the propagation of electrical nerve impulses, with eventual absence or profound delay in conduction, causing flaccid paralysis. Recovery is typically associated with remyelination. […] In some patients with severe disease, a secondary consequence of the severe inflammation is axonal disruption and loss. A subgroup of patients may have a primary immune attack directly against nerve axons, with sparing of myelin. The clinical presentation in these patients is similar to that of the principal type.

• #16 Guillain-Barre Syndrome Workup: Approach Considerations, Peripheral Neuropathy Workup, Biochemical Screening

  https://emedicine.medscape.com/article/315632-workup

  Lymphocyte and macrophage infiltration is observed on microscopic examination of peripheral nerves, with macrophage influx believed to be responsible for the multifocal demyelination seen in GBS. A variable degree of wallerian degeneration also can be observed with severe inflammatory changes. […] Cellular infiltrates are scattered throughout the cranial nerves, nerve roots, dorsal root ganglions, and peripheral nerves.

• #17

  https://link.springer.com/article/10.1007/s40120-021-00261-4

  The hypothesis of molecular mimicry has not been clearly demonstrated in AIDP. […] However, antecedent infection and deposition of complement proteins in biopsied or autopsied peripheral nerve specimens suggest that mechanisms similar to those in AMAN also participate in AIDP. […] Some investigators have suggested that mechanisms resulting from molecular mimicry between viral proteins and human proteins participate in the pathogenesis of Zika/COVID-19-associated GBS. […] It has been suggested that exacerbation of COVID-19 is triggered by an excessive host immune response induced by SARS-CoV-2. […] Some investigators have suggested that additional autoimmune mechanisms resulting from molecular mimicry between SARS-CoV-2 surface proteins and human proteins participate in the pathogenesis of complications associated with SARS-CoV-2 infection, such as acute respiratory distress syndrome and GBS.

• #18 Pulsenotes | Guillain-Barré syndrome notes

  https://app.pulsenotes.com/medicine/neurology/notes/guillain-barre-syndrome

  Guillain-Barr syndrome is an acute, inflammatory polyneuropathy typically characterised by a progressive, ascending neuropathy resulting in weakness and reduced reflexes. […] GBS is an umbrella term that refers to a number of variants with unique features and pathogenesis. […] GBS involves immune-mediated damage to peripheral nerves. One proposed mechanism is molecular mimicry where antibodies generated in response to pathogens incorrectly cross-react with the bodies own cells. […] The pattern of nerve involvement varies between variants of GBS. The myelin sheath is the target in AIDP (though the axon may be affected) whilst in AMAN and AMSAN axonal degeneration occurs. […] AIDP is caused by immune-mediated inflammatory demyelination of peripheral nerves. […] AMAN is a form of GBS that results from axonal involvement typically following infection with Campylobacter jejuni. […] A severe form of AMAN that also demonstrates involvement of sensory nerves. It is characterised by axonal degeneration of both sensory and peripheral nerves.

• #19 Axonal pathology in early stages of Guillain-Barré syndrome | Neurología (English Edition)

  https://www.elsevier.es/es-revista-neurologia-english-edition–495-articulo-axonal-pathology-in-early-stages-S2173580820301553

  Guillain-Barr syndrome (GBS) is an acute-onset, immune-mediated disease of the peripheral nervous system. It may be classified into 2 main subtypes: demyelinating (AIDP) and axonal (AMAN). This study aims to analyse the mechanisms of axonal damage in the early stages of GBS (within 10 days of onset). […] Inflammatory oedema of the spinal nerve roots and spinal nerves is the initial lesion in GBS. The spinal nerves of patients with fatal AIDP may show ischaemic lesions in the endoneurium, which suggests that endoneurial inflammation may increase endoneurial fluid pressure, reducing transperineurial blood flow, potentially leading to conduction failure and eventually to axonal degeneration. […] In patients with AMAN associated with anti-ganglioside antibodies, nerve conduction block secondary to nodal sodium channel dysfunction may affect the proximal, intermediate, and distal nerve trunks. In addition to the mechanisms involved in AIDP, active axonal degeneration in AMAN may be associated with nodal axolemma disruption caused by anti-ganglioside antibodies.

• #20 Guillain-Barré Syndrome | AAFP

  https://www.aafp.org/pubs/afp/issues/2004/0515/p2405.html

  Guillain-Barr syndrome (GBS) is a group of autoimmune syndromes consisting of demyelinating and acute axonal degenerating forms of the disease. […] Cross-reactivity between the pathogen and the nerve tissue sets up the autoimmune response. […] Acute infectious illnesses are well-known antecedent events in two thirds of patients who have GBS. Cross-reactivity between the pathogen and the nerve tissue sets up the autoimmune response. […] Electrophysiologic classification demonstrated that the subtypes AMAN and AMSAN occurred more frequently in the C. jejuni-infected patients with GBS than the subtype AIDP. C. jejuni infection is associated with slower recovery, axonal degeneration, and severe residual disability. […] Specific treatment should be initiated soon after diagnosis. High-dose intravenous immunoglobulin (IVIg; 400 mg per kg daily for five days) or plasmapheresis (five exchanges over five to eight days) can be initiated.

• #21 Davidson College: Immune Condition: Guillain-Barre Syndrome

  https://www.bio.davidson.edu/movies/Immunology/Students/spring2006/Blumer/GBS.html

  The strongest evidence for the molecular mimicry hypothesis has come from discoveries in research with C. jejuni strains, the most common pathogen associated with GBS (specifically AMAN) (Neisser et al., 2000; Yuki, 2005). […] The hypothesis of molecular mimicry is based on the fact that the bacterial LOS induces IgG, IgA, and IgM autoantibodies against human gangliosides due to LOS ganglioside-mimicking epitopes (Figure 4) (Moran et al., 2005). Autoantibodies have been isolated in GBS patients’ serum and found to recognize C. jejuni LOS and human gangliosides GM1, GM1b, GD1a, and GalNAc-GD1a epitopes, providing evidence for molecular mimicry (Ang et al., 2004; Yuki, 2005). […] Infection by C. jejuni is also implicated in the development of MFS by the same mechanism of molecular mimicry.

• #22 Axonal pathology in early stages of Guillain-Barré syndrome | Neurología (English Edition)

  https://www.elsevier.es/es-revista-neurologia-english-edition–495-articulo-axonal-pathology-in-early-stages-S2173580820301553

  Inflammatory oedema of the proximal nerve trunks can be observed in early stages of GBS, and it may cause nerve conduction failure and active axonal degeneration. […] The blood-nerve barrier plays an essential role in the homeostasis of the PNS. […] These characteristics of the blood-nerve barrier explain the selective vulnerability of the spinal roots, spinal ganglia, spinal nerves, and nerve terminals to autoimmune attacks, particularly those mediated by antibodies. […] The concept of nodo-paranodopathies enables novel pathogenic perspectives, which are of great importance when interpreting the neurophysiological alterations occurring in GBS. […] Early GBS involves inflammatory oedema of proximal nerve trunks, which may lead to nerve conduction failure and active axonal degeneration.

• #23

  https://link.springer.com/article/10.1007/s13311-021-01117-3

  Classical CIDP pathology is associated with macrophage and T cell infiltration in the peripheral nerves and nerve roots that results in segmental demyelination. […] It is unclear how blood-nerve (and blood-brain) barrier breakdown happens in CIDP, but, at least in animal models, it is one of the first events in nerve inflammation and may be mediated by inflammatory molecules released by inflammatory cells in the peripheral immune compartment. […] Demyelination caused by macrophages has been proposed to play an important role in the pathogenesis of CIDP: macrophage infiltration of the nerves triggers myelin breakdown through phagocytosis. […] Activated circulating T lymphocytes, elevated serum levels of pro-inflammatory cytokines, and the presence of CD4+ and CD8+ T cells infiltrating sural nerve biopsies suggest an important role for T cells in CIDP development as well.

• #24 The Immunopathogenesis of Guillain-Barré Syndrome – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/march-2010/the-immunopathogenesis-of-guillain-barre-syndrome/

  The pathogenesis of GBS is unclear, although there is strong evidence of immune dysfunction with delayed hypersensitivity to components of peripheral nervous system myelin. In AIDP, macrophages invade intact myelin sheaths. This has been postulated to be due to a T-cell mediated process against one of the myelin proteins, as seen in the animal model of GBS-experimental allergic neuritis; a secondary enhancement of demyelination by an antibody-mediated process may be involved. Alternatively the initial event may be binding of antibodies to the Schwann cell surface and demyelination, perhaps prior to the cell invasion by macrophages. On the other hand, in AMAN, there is strong evidence to support axonal destruction by antibodies to gangliosides on the nerve axolemma that target macrophages to invade the axon at the node of Ranvier as the result of molecular mimicry.

• #25 Clinical efficacy and mechanism of lymphoplasma exchange in the treatment of Guillain-Barre syndrome | Cellular and Molecular Biology

  https://www.cellmolbiol.org/index.php/CMB/article/view/1576

  Guillain-Barre syndrome (GBS) is an autoimmune disease of the nervous system and is the most common acute polyneuropathy. Both cellular and humoral immunity are believed to be involved in the pathogenesis of GBS, and various types of activated CD4+ T cells are thought to orchestrate the onset and progression of GBS. […] Our data indicate that LPE is effective in treating GBS patients by directly removing immunoglobulin, complement, monocytes, and fibrinogen as well as regulating lymphocyte subsets in the peripheral blood.

• #26 Guillain-Barrв€љВ© & Charcot-Marie-Tooth – Free Sketchy Medical Lesson

  https://www.sketchy.com/medical-lessons/guillain-barre-charcot-marie-tooth

  Guillain-Barr syndrome (GBS) is an immune-mediated demyelinating disorder targeting the peripheral nervous system, sometimes referred to as acute inflammatory demyelinating polyradiculoneuropathy. GBS may be caused by an inappropriate immune response following an infection, resulting in molecular mimicry, where the antigen of the pathogen cross-reacts with components of the peripheral nerves. Schwann cells, the main producers of myelin in the peripheral nerves, are the primary target of this autoimmune response. […] The pathogenesis of GBS incorporates both cellular and humoral immune responses. The cellular immune response includes the activation of helper T-cells that recruit macrophages, while a humoral response likely involves the autoantibody-mediated destruction of Schwann cells or myelin antigens. Additionally, local activation of the complement cascade from antibody binding may also play a role.

• #27 Toll-like receptor 2 and -4 are involved in the pathogenesis of the Guillain-Barré syndrome

  https://www.spandidos-publications.com/10.3892/mmr.2015.3730

  The current hypothesis states that TLRs are involved in the initiation of GBS. Following TLR activation, they increase NF-B levels through MyD88-dependent or -independent pathways, and finally induce the secretion of pro-inflammatory cytokines. […] GBS arises as a result of an autoimmune attack due to structural similarities between certain LPS molecules from Campylobacter jejuni and human nerve tissue gangliosides. […] Excessive activation of TLRs is capable of eliminating auto-immune tolerance and may lead to autoimmune diseases, including systemic lupus erythematosus (SLE) and autoimmune hepatitis. […] The present study identified that TLR2 and TLR4 and their signaling pathways are functionally involved in the pathogenesis of GBS. […] The present study suggested that TLR2, TLR4 and their signaling pathways may be potential treatment targets, which requires further investigation.

• #28 Investigating Guillain-Barre Syndrome and Vaccines: Is There a Link?

  https://www.neurologyadvisor.com/features/investigating-guillain-barre-syndrome-and-vaccines-is-there-a-link/

  The rare neurologic disorder, GBS, occurs when the body attacks its nerves due to immune reactions triggered by certain bacterial or viral infections. It typically occurs in 0.4 to 4.0 cases per 100,000 individuals, with higher rates seen in men aged 75 years and older. […] When a virus triggers an immune reaction, the frontline immune cells of the body will activate T cell differentiation, which triggers macrophages and B cells. Macrophages secrete matrix metalloproteinases (MMPs) and nitric oxide; B cells produce immunoglobulin G (IgG) to eradicate the virus, but when these proteins and immunoglobulins leak through the blood-nerve barrier, this mechanism can damage the peripheral nervous system. IgG can also trigger the complement system which can result in further nerve cell damage. […] However, regarding vaccine-associated GBS, the mechanism could potentially involve similar immune patterns during vaccine-induced autoimmunity.

• #29

  https://www.archivesofmedicalscience.com/Pathogenic-mechanism-of-intestinal-microbiota-involved-in-Guillain-Barre-syndrome,128103,0,2.html

  Guillain-Barr syndrome (GBS) is an acute immune-mediated polyneuropathy, involving the peripheral nervous system. The pathogenicity of GBS involves Th17/Treg imbalance, which may be affected by intestinal microbiota. […] In the present study, we aimed to further investigate the pathogenic mechanisms of intestinal microbiota involved in GBS, with the intervention of Bifidobacterium. […] Imbalance of Th17/Tregs expression induced by imbalance of intestinal microbiota may get involved in GBS. However, B. infantis intervention can regulate Th17/Treg balance by regulating imbalanced intestinal microbiota, thus improving GBS.

• #30 Guillain-Barré syndrome pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Guillain-Barr%C3%A9_syndrome_pathophysiology

  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. […] It seems that antibody-mediated mechanism is more prominent in AMAN, whereas cellular mechanisms are more important in AIDP.

• #31 Molecular Mimicry in Guillain-Barre Syndrome – Creative Med Doses

  https://creativemeddoses.com/topics-list/molecular-mimicry-in-guillain-barre-syndrome/

  Molecular Mimicry is the condition where immune system gets confused between self and non-self antigens due to molecular similarities between an environmental agent (bacteria and virus most commonly) and the host. Molecular mimicry can explain pathogenesis of few autoimmune diseases and this post is about Guillain-Barre Syndrome (GBS). […] Guillain-Barre Syndrome is characterized by an immune-mediated attack on peripheral nerve, more commonly in the myelin sheath or Schwann cells of sensory and motor nerves. […] Infection with few pathogens leads to the production of antibodies to a carbohydrate-containing surface antigen that cross-reacts with the myelin sheath and the axons of nerve cells, resulting in demyelination and axonal degeneration. […] Antibody and/or cell-mediated immune responses are involved in degeneration of the nerve and interruption of neurotransmission which is responsible for muscle weakness. […] Approximately 70-80% of cases of GBS occur few weeks after an acute infection, usually respiratory or gastrointestinal. […] Similar antigens—Confusion between self and non-self—attack on myelin sheath —-impaired neurotransmission—-muscle weakness—-GBS

• #32 Guillain-Barré Syndrome : Emergency Care BC

  https://emergencycarebc.ca/clinical_resource/clinical-summary/guillain-barre-syndrome/

  Guillain-Barré Syndrome (GBS) is an acute polyneuropathy characterized by immune-mediated peripheral nerve myelin sheath or axon destruction usually in the setting of recent infection. […] Cross-reactive antibodies are formed in response to this prior viral or bacterial illness and result in an autoimmune response directed against the myelin sheath and axons of peripheral nerves. […] Campylobacter jejuni is the most common infectious agent associated with GBS. […] Cytomegalovirus (CMV), Epstein-Barr virus (EBV), and Mycoplasma pneumoniae are also associated with development of GBS.

• #33 Do Vaccines Cause Guillain-Barré Syndrome? – Institute for Vaccine Safety

  https://www.vaccinesafety.edu/do-vaccines-cause-guillain-barre-syndrome/

  Most GBS cases are preceded by a recent respiratory or gastrointestinal infection. Campylobacter jejuni, which causes gastrointestinal infections, is the most common specific infectious agent identified through molecular mimicry. Campylobacter jejuni induces antibodies that react against GM1 gangliosides in human neurons due to shared antigenic and epitopic features with lipo-oligosaccharide moieties on the cell wall of the Campylobacter bacterium. […] The mechanism for other infectious agents associated with GBS has not been identified.

• #34

  https://link.springer.com/article/10.1007/s40120-021-00261-4

  GuillainBarr syndrome (GBS) is an autoimmune disorder of the peripheral nervous system that typically develops within 4 weeks after infection. […] The concept of molecular mimicry between pathogens and human peripheral nerve components was established through studies of AMAN with anti-ganglioside GM1 antibodies occurring after Campylobacter jejuni infection. […] Although such mimicry between specific pathogens and myelin or Schwann cell components has not been clearly demonstrated in AIDP, a similarity of Zika virus and SARS-CoV-2 proteins to human proteins has been suggested. […] The production of autoantibodies directed against peripheral nerve components, particularly gangliosides, induced by infection with these pathogens is considered to play an important role in the pathogenesis of GBS.

• #35 Guillain–Barre syndrome: demographics, clinical features, and outcome in a single tertiary care hospital, Oman | Neurosciences Journal

  https://nsj.org.sa/content/25/5/369

  GuillainBarre syndrome (GBS) is an acute autoimmune-mediated peripheral nervous system disease. […] The syndrome is characterized by an acute or subacute progressive generalized muscle weakness of upper and lower limbs with relative or complete areflexia, and limb paraesthesia. […] Proceeding infections (such as infections with Campylobacter jejuni, Cytomegalovirus, EpsteinBarr virus, Mycoplasma pneumonia, and HIV) may activate the immune response, owing to molecular mimicry, to cross-react with the peripheral nerves components (the myelin and/or the axon) resulting in the endoneural inflammation of spinal nerve roots, nerves distal segments, and at potential nerve entrapments sites. […] Some triggering factors (e.g., immunization, trauma, bone marrow transplantation, and surgery) may also affect the pathogenesis of GBS.

• #36 Guillain-Barré Syndrome vs. Myasthenia Gravis

  https://ameripharmaspecialty.com/myasthenia-gravis/guillain-barre-syndrome-vs-myasthenia-gravis/

  Guillain-barré syndrome is caused by the immune system attacking the peripheral nerves, which are located outside the brain and control movement and sensation. The immune system produces antibodies that mistakenly target the myelin sheath (a protective covering around the nerves) or the nerve fibers themselves, leading to nerve damage and inflammation. This damage disrupts the transmission of signals from the brain to the muscles and results in muscle weakness and paralysis. […] The exact cause of GBS is still unknown, but researchers believe it is triggered by an infection or other illnesses. In many cases, GBS develops a few days or weeks after a respiratory or gastrointestinal infection. […] Other factors that can trigger the onset of GBS include; Zika virus and COVID-19, vaccination, surgery, or trauma.

• #37

  https://journals.lww.com/md-journal/fulltext/2024/07260/clinical_presentation_and_symptomatology_of.28.aspx

  The prevailing hypothesis implicates molecular mimicry, wherein microbial antigens share structural similarities with components of peripheral nerve tissues, triggering an autoimmune attack. […] This autoimmune assault manifests as inflammation and subsequent demyelination or axonal damage, leading to the hallmark motor and sensory deficits observed in GBS. […] Infections, particularly bacterial and viral pathogens, serve as common precipitants of GBS, with Campylobacter jejuni, cytomegalovirus (CMV), Epstein-Barr virus (EBV), and Zika virus among the most frequently implicated culprits. […] The pathophysiological journey of GBS extends beyond the peripheral nervous system, with emerging evidence implicating dysregulation of the blood-nerve barrier and activation of innate immune pathways in disease progression. […] Additionally, genetic predisposition may influence susceptibility to GBS, with certain human leukocyte antigen (HLA) alleles and polymorphisms in immune-related genes identified as potential risk factors.

• #38 Guillain-Barré Syndrome | AAFP

  https://www.aafp.org/pubs/afp/issues/2004/0515/p2405.html

  Plasmapheresis removes or dilutes the circulating immune factors implicated in the pathogenesis of GBS. […] Modulation of the immune system is thought to occur through multiple mechanisms involving the constant and variable regions of immunoglobulin class G (IgG), as well as receptors on macrophages and B cells. Pathologic antibodies might be bound by the IgG increasing their clearance.

• #39 UNDERSTANDING THE MECHANISM OF ACTION OF INTRAVENOUS IMMUNOGLOBULINS: A TEN YEARS EXPERIENCE IN TREATING GUILLAIN BARRÉ SYNDROME – Farmacia Journal

  https://farmaciajournal.com/issue-articles/understanding-the-mechanism-of-action-of-intravenous-immunoglobulins-a-ten-years-experience-in-treating-guillain-barre-syndrome/

  Guillain Barr syndrome is an acute immune-mediated, heterogeneous disease that affects the peripheral nervous system and is characterized by rapidly progressive motor deficit, diminished reflexes, cranial nerve involvement, respiratory failure, sometimes with involvement of autonomic nervous system which contribute to the mortality rate. […] Both T-cells and B-cells are involved in its pathogenesis, finally leading to myelin destruction or axonal damage. […] The present paper underlines the effect of intravenous immunoglobulins in treating this disease by discussing the pathophysiology of Guillain Barr syndrome with the participation of immune system and the mechanism of action of intravenous immunoglobulins with the reduction of autoantibodies, down regulation of macrophage activity, reduction of inflammatory cytokine levels and cell adhesion molecules thus limiting the self-harm and the passage of autoimmune cells through the blood-nerve barrier.

• #40 Prognostic factors of Guillain-Barré syndrome: a 111-case retrospective review | Chinese Neurosurgical Journal | Full Text

  https://cnjournal.biomedcentral.com/articles/10.1186/s41016-018-0122-y

  Guillain-Barre syndrome (GBS) is a set of clinical syndromes with a common pathophysiological basis, and is usually considered to be an immune-mediated disorder of the peripheral nervous system. […] GBS is usually self-limiting, and most patients either recover completely or only retain minor residual symptoms. But there are still several patients who may face severe outcomes including death. […] Diabetes, high fasting blood glucose level and high blood pressure at admission, uroschesis, abnormal body temperature, requiring ventilator support, disorder of consciousness, no preceding upper respiratory tract infection, low level of blood sodium and albumin, high white blood cell count, high fibrinogen level, and abnormal hepatic and renal function were demonstrated as poor prognostic factors.

• #41 Prognostic factors of Guillain-Barré syndrome: a 111-case retrospective review | Chinese Neurosurgical Journal | Full Text

  https://cnjournal.biomedcentral.com/articles/10.1186/s41016-018-0122-y

  The infectious event is described to appear in 40-70% of patients. […] In our study, we discovered that MFS accounted for 33.33% patients among those who had preceding upper respiratory infection, and only 11.90% patients among those who didn’t have preceding infection. […] In our study, patients with diabetes and high fasting blood glucose had poorer prognosis. […] GBS has a tendency for dysautonomic features such as bladder dysfunction, abnormal body temperature and hypertension among patients. […] Hyponatremia is so common in GBS patient, despite that it is not a classical manifestation of GBS; however, there are series in which are described to be present in 21.5 to 48% of the cases; in our review, it was found in 21.6% of our patients, which is similar to the Northern China study and the British study.

• #42 Prognostic factors of Guillain-Barré syndrome: a 111-case retrospective review | Chinese Neurosurgical Journal | Full Text

  https://cnjournal.biomedcentral.com/articles/10.1186/s41016-018-0122-y

  Our study also found elevated liver enzyme level indicates poorer prognosis as well. […] According to Khajehdehi P et al’s research, acute renal failure can occur commonly in cases with severe GBS patients particularly in those with dysautonomia. […] Our study also found that poor nutrition condition such as low serum albumin level, high coagulation state as high fibrinogen level, and infection at early stage of GBS such as high white blood cell count, are also predictors of poorer prognosis.

• #43 Advances in clinical approach of Guillain-Barré syndrome – ean.org

  https://www.ean.org/research/resources/neurology-updates/detail/advances-in-clinical-approach-of-guillain-barre-syndrome

  Current developments have refined the diagnostic framework for Guillain-Barr Syndrome (GBS). […] This review highlights the need to update our understanding of GBS and its pathogenesis. […] Improved understanding of the pathogenesis and biomarkers is essential for better diagnostics and therapeutic approach.

• #44 Autoimmune mechanisms in Guillain-Barré syndrome subtypes: a systematic review | Bulletin of Faculty of Physical Therapy | Full Text

  https://bfpt.springeropen.com/articles/10.1186/s43161-024-00258-8

  Complement inhibitors, such as eculizumab, offer promising therapeutic strategies, particularly for axonal forms of GBS. […] This study has explored the autoimmune mechanisms underlying GBS, emphasizing the immune-mediated attack on the peripheral nervous system. Key findings highlight the roles of molecular mimicry, autoantibodies, and the activation of both innate and adaptive immune responses.

• #45 Guillain-Barré Syndrome: researchers find cause • healthcare-in-europe.com

  https://healthcare-in-europe.com/en/news/guillain-barre-syndrome-research-cause.html

  However, in rare cases, they can mistakenly attack the body’s own tissues, leading to autoimmune diseases. […] Furthermore, the researchers identified T lymphocytes reactive to both self-antigens of peripheral nerves (myelin) and viral antigens in a subset of post-viral GBS patients, supporting a direct link between disease development and triggers of a preceding infection. […] Overall, the work of the research team offers novel insights into our understanding of GBS, opening avenues for further investigations on larger patient groups to decipher immune mechanisms in different GBS variants.

Zobacz więcej