Guillain-Barre Syndrome

name: Guillain-Barre Syndrome
creation_date: '2025-12-19T01:12:52Z'
updated_date: '2026-02-17T21:53:14Z'
category: Autoimmune
parents:
- Autoimmune Disease
- Peripheral Neuropathy
disease_term:
  preferred_term: Guillain-Barre Syndrome
  term:
    id: MONDO:0016218
    label: Guillain-Barre syndrome
description: >-
  An acute immune-mediated polyradiculoneuropathy characterized by rapidly
  progressive symmetric weakness and areflexia. Typically preceded by infection,
  with molecular mimicry between pathogen antigens and peripheral nerve
  gangliosides driving the autoimmune response.
pathophysiology:
- name: Molecular Mimicry and Antiganglioside Antibodies
  description: >-
    Antibodies against bacterial lipooligosaccharides (especially from
    Campylobacter jejuni) cross-react with gangliosides on peripheral nerves.
    Anti-GM1, anti-GD1a, and anti-GQ1b antibodies are associated with
    specific clinical subtypes.
  biological_processes:
  - preferred_term: Immunoglobulin Production
    term:
      id: GO:0002377
      label: immunoglobulin production
  evidence:
  - reference: PMID:24000328
    reference_title: "Role of Campylobacter jejuni infection in the pathogenesis of Guillain-Barré syndrome: an update."
    supports: SUPPORT
    snippet: >-
      Molecular mimicry between sialylated lipooligosaccharide structures on the cell
      envelope of these bacteria and ganglioside epitopes on the human nerves that
      generates cross-reactive immune response results in autoimmune-driven nerve
      damage.
    explanation: >-
      Demonstrates the molecular mimicry mechanism where C. jejuni lipooligosaccharides
      resemble nerve gangliosides, leading to cross-reactive antibodies that attack
      peripheral nerves.
  - reference: PMID:37108447
    reference_title: "Immune-Mediated Neuropathies: Pathophysiology and Management."
    supports: SUPPORT
    snippet: >-
      The presence of specific anti-glycoconjugate antibodies indicates an underlying
      process of molecular mimicry and sometimes assists in the classification of
      these
      disorders.
    explanation: >-
      Confirms that anti-ganglioside antibodies in GBS result from molecular mimicry
      and help classify disease subtypes.
- name: Complement-Mediated Nerve Damage
  description: >-
    Antibody binding activates complement cascade, leading to membrane attack
    complex deposition on Schwann cells and nodes of Ranvier. This causes
    demyelination in AIDP or axonal damage in AMAN/AMSAN variants.
  cell_types:
  - preferred_term: Schwann Cell
    term:
      id: CL:0002573
      label: Schwann cell
  biological_processes:
  - preferred_term: Complement Activation
    term:
      id: GO:0006956
      label: complement activation
  evidence:
  - reference: PMID:10355667
    reference_title: "Anti-ganglioside antibodies can bind peripheral nerve nodes of Ranvier and activate the complement cascade without inducing acute conduction block in vitro."
    supports: PARTIAL
    snippet: >-
      These data indicate that anti-ganglioside antibodies can diffuse into a
      desheathed nerve, bind to nodes of Ranvier and fix complement in vitro without
      resulting in any overt physiological deterioration of the nerve over 4-6 h.
    explanation: >-
      Demonstrates that anti-ganglioside antibodies bind nodes of Ranvier and activate
      complement cascade, establishing the complement-mediated mechanism of nerve
      damage.
  - reference: PMID:8619548
    reference_title: "Immune attack on the Schwann cell surface in acute inflammatory demyelinating polyneuropathy."
    supports: SUPPORT
    snippet: >-
      Many fibers had a rim of the complement activation marker C3d and the terminal
      complement complex neoantigen C5b-9 along the outer surface of the Schwann cells.
    explanation: >-
      Immunocytochemistry evidence showing C3d and C5b-9 membrane attack complex deposition
      on Schwann cell surfaces in AIDP, confirming complement-mediated pathology.
- name: Macrophage-Mediated Demyelination
  description: >-
    In AIDP, macrophages infiltrate peripheral nerves and strip myelin from
    axons. T cells may also contribute to the inflammatory milieu and
    tissue damage.
  cell_types:
  - preferred_term: Macrophage
    term:
      id: CL:0000235
      label: macrophage
  biological_processes:
  - preferred_term: Inflammatory Response
    term:
      id: GO:0006954
      label: inflammatory response
  evidence:
  - reference: PMID:37108447
    reference_title: "Immune-Mediated Neuropathies: Pathophysiology and Management."
    supports: SUPPORT
    snippet: >-
      The immunological mechanisms, which include macrophage infiltration, inflammation
      and proliferation of Schwann cells, result in variable degrees of demyelination
      and axonal degeneration.
    explanation: >-
      Describes macrophage infiltration as a key immunological mechanism leading to
      demyelination and axonal degeneration in immune-mediated neuropathies including
      GBS.
  - reference: PMID:8619548
    reference_title: "Immune attack on the Schwann cell surface in acute inflammatory demyelinating polyneuropathy."
    supports: SUPPORT
    snippet: >-
      Vesicular degeneration was seen before the invasion of macrophages into the
      myelin,
      and was the predominant change in the subject with symptoms for 3 days.
    explanation: >-
      Shows the temporal sequence of pathology in AIDP, with macrophage invasion into
      myelin following initial complement-mediated damage.
phenotypes:
- name: Areflexia
  category: Neurological
  frequency: VERY_FREQUENT
  phenotype_term:
    preferred_term: Areflexia
    term:
      id: HP:0001284
      label: Areflexia
  notes: Universal finding
- name: Limb Weakness
  category: Neurological
  frequency: VERY_FREQUENT
  phenotype_term:
    preferred_term: Limb Muscle Weakness
    term:
      id: HP:0003690
      label: Limb muscle weakness
  notes: Ascending, symmetric
- name: Respiratory Failure
  category: Respiratory
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Respiratory Insufficiency
    term:
      id: HP:0002093
      label: Respiratory insufficiency
  notes: May require mechanical ventilation
- name: Paresthesia
  category: Neurological
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Paresthesia
    term:
      id: HP:0003401
      label: Paresthesia
  notes: Sensory symptoms often precede weakness
biochemical:
- name: CSF Protein
  presence: Elevated
  context: Albuminocytologic dissociation characteristic
- name: Antiganglioside Antibodies
  presence: Variable
  context: GM1, GD1a, GQ1b depending on subtype
environmental:
- name: Campylobacter jejuni Infection
  notes: Most common preceding infection
  evidence:
  - reference: PMID:24000328
    reference_title: "Role of Campylobacter jejuni infection in the pathogenesis of Guillain-Barré syndrome: an update."
    supports: SUPPORT
    snippet: >-
      Thus, C. jejuni is now considered as a major triggering agent of GBS. Molecular
      mimicry between sialylated lipooligosaccharide structures on the cell envelope
      of these bacteria and ganglioside epitopes on the human nerves that generates
      cross-reactive immune response results in autoimmune-driven nerve damage.
    explanation: >-
      Establishes C. jejuni as the major triggering infection for GBS through molecular
      mimicry mechanism between bacterial lipooligosaccharides and nerve gangliosides.
- name: Cytomegalovirus Infection
  notes: Associated with sensory GBS
- name: Zika Virus Infection
  notes: Associated with outbreaks
treatments:
- name: IVIG
  description: First-line immunotherapy, equally effective as plasmapheresis.
- name: Plasmapheresis
  description: Removes pathogenic antibodies; alternative to IVIG.
- name: Supportive Care
  description: ICU monitoring, mechanical ventilation if needed.
- name: Physical Therapy
  description: Rehabilitation during recovery phase.
classifications:
  harrisons_chapter:
  - classification_value: nervous system disorder
  - classification_value: neuromuscular disease
  - classification_value: autoimmune disease
references:
- reference: DOI:10.1007/s00415-024-12186-7
  title: 'Guillain–Barré syndrome and COVID-19 vaccination: a systematic review and
    meta-analysis'
  findings: []
- reference: DOI:10.1038/s41598-024-74729-2
  title: Global burden of vaccine-associated Guillain-Barré syndrome over 170
    countries from 1967 to 2023
  findings: []
- reference: DOI:10.1093/brain/awac418
  title: Anti-pan-neurofascin antibodies induce subclass-related complement
    activation and nodo-paranodal damage
  findings: []
- reference: DOI:10.1111/jns.12625
  title: 'Conduction slowing, conduction block and temporal dispersion in demyelinating,
    dysmyelinating and axonal neuropathies: Electrophysiology meets pathology'
  findings: []
- reference: DOI:10.1186/s43161-024-00258-8
  title: 'Autoimmune mechanisms in Guillain-Barré syndrome subtypes: a systematic
    review'
  findings: []
- reference: DOI:10.3389/fimmu.2024.1415986
  title: 'Variation in worldwide incidence of Guillain-Barré syndrome: a population-based
    study in urban China and existing global evidence'
  findings: []
- reference: DOI:10.3390/ijms24087288
  title: 'Immune-Mediated Neuropathies: Pathophysiology and Management'
  findings: []
- reference: DOI:10.69622/28457924.v1
  title: Biomarker and pathogenic study of immune-mediated neuropathies
  findings: []