Acute Motor and Sensory Axonal Neuropathy

Autoimmune MONDO:0020348 Pathograph 12 Guillain-Barre Syndrome Peripheral Neuropathy

Acute motor and sensory axonal neuropathy (AMSAN) is an acute immune-mediated axonal variant of Guillain-Barre syndrome with motor and sensory peripheral nerve involvement. It is typically considered a post-infectious autoimmune neuropathy in which anti-ganglioside antibodies and complement-mediated nodal or axonal injury contribute to rapidly progressive weakness, sensory symptoms, and areflexia.

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5
Pathophys.
7
Phenotypes
12
Pathograph
1
Genes
4
Treatments
1
Trials
10
References
1
Deep Research
⚙

Pathophysiology

5
Post-Infectious Molecular Mimicry
Campylobacter jejuni infection can trigger antibodies that cross-react with peripheral nerve gangliosides through molecular mimicry, providing an upstream immune mechanism for axonal GBS variants including AMSAN.
B cell lineage lymphocyte link
Adaptive immune response link Immunoglobulin production link
Downstream
Ganglioside Antibody Binding at Nodes of Ranvier Infection-associated molecular mimicry produces anti-ganglioside antibodies that can bind peripheral nerve ganglioside targets.
Show evidence (2 references)
DOI:10.3390/microorganisms10112139 SUPPORT Human Clinical
"GBS is most often of the axonal subtype and is thought to be mediated by IgG antibodies to peripheral nerve gangliosides that are cross reactive with oligosaccharides in the Cj lipopolysaccharides (LPS)."
This review links axonal GBS to anti-ganglioside IgG induced by Campylobacter jejuni molecular mimicry, supporting the upstream immune mechanism applied to AMSAN as an axonal GBS variant.
DOI:10.3390/microorganisms10112139 SUPPORT Human Clinical
"The antibodies are thought to be induced by molecular mimicry, where immune reactivity to a cross reactive epitope in the infectious organism and normal tissue can cause autoimmune disease."
The abstract explicitly states the molecular-mimicry model that explains how an antecedent infection can produce cross-reactive autoimmunity.
Ganglioside Antibody Binding at Nodes of Ranvier
Anti-ganglioside antibodies bind ganglioside targets on peripheral nerve fibers, especially nodal and axolemmal regions implicated in axonal GBS variants.
Neuron link
Immune response link
Downstream
Complement Activation at Axolemma Ganglioside-bound antibodies can activate complement on peripheral nerve membranes.
Show evidence (2 references)
PMID:37090048 SUPPORT Human Clinical
"gangliosides can activate complement system and recruit macrophages on the"
This supports antibody binding to gangliosides as the upstream interaction in ganglioside-antibody mediated GBS.
PMID:22507308 SUPPORT Human Clinical
"Serum antibodies to different gangliosides have been identified in some"
The review supports antiganglioside antibodies as a shared immune feature across GBS subtypes and variants, including axonal variants.
Complement Activation at Axolemma
Complement-fixing anti-ganglioside antibodies activate complement at peripheral nerve axolemmal and nodal regions. Complement activation is modeled as a distinct effector step upstream of immune-cell recruitment and axonal injury.
Neuron link
Complement activation link
Downstream
Macrophage Recruitment to Complement Deposition Sites Complement activation and deposition recruit immune cells to affected peripheral nerve sites.
Axonal Degeneration and Conduction Failure Complement deposition is upstream of immune-cell recruitment and axonal injury in the acute axonal GBS model.
Show evidence (2 references)
PMID:37090048 SUPPORT Human Clinical
"gangliosides can activate complement system and recruit macrophages on the"
This supports complement activation as a consequence of anti-ganglioside antibody binding in GBS.
PMID:26936605 PARTIAL Model Organism
"attenuated complement cascade activation and deposition, reduced immune cell"
This animal-model evidence supports complement activation and deposition as a mechanistic step in acute axonal GBS models; it is relevant to AMSAN mechanism but is not direct human AMSAN evidence.
Macrophage Recruitment to Complement Deposition Sites
Complement deposition at peripheral nerve membranes recruits macrophages and other immune cells to the injury site; this node captures recruitment rather than antibody binding or axonal degeneration.
Macrophage link
Macrophage chemotaxis link
Downstream
Axonal Degeneration and Conduction Failure Recruited immune cells contribute to peripheral nerve axonal injury.
Show evidence (2 references)
PMID:37090048 SUPPORT Human Clinical
"gangliosides can activate complement system and recruit macrophages on the"
This supports macrophage recruitment downstream of ganglioside-antibody interactions in GBS.
PMID:26936605 PARTIAL Model Organism
"attenuated complement cascade activation and deposition, reduced immune cell"
This model-organism evidence links complement inhibition to reduced immune cell recruitment in an acute axonal GBS model.
Axonal Degeneration and Conduction Failure
Axonal injury in AMSAN produces sensory-motor peripheral nerve dysfunction, including reduced conduction, progressive limb weakness, sensory symptoms, and neurophysiologic evidence of an acute motor-sensory axonal GBS pattern.
Neuron link
Downstream
Limb Weakness Motor axonal dysfunction causes progressive limb weakness.
Sensory Neuropathy Sensory axonal dysfunction causes sensory neuropathy and paresthesia.
Neurophysiology and CSF Evaluation The downstream axonal injury state is detected by neurophysiologic testing used in AMSAN diagnosis.
Show evidence (2 references)
PMID:26936605 PARTIAL Model Organism
"attenuated complement cascade activation and deposition, reduced immune cell"
This animal-model evidence supports axonal injury as a downstream consequence of complement-mediated acute axonal GBS mechanisms.
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"Following a neuro-electro-physiology study to evaluate neurological symptoms, AMSAN was suggested."
This AMSAN case report supports neurophysiologic evidence of the downstream acute motor-sensory axonal neuropathy state.
⬡

Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Referential integrity issues (1):
  • Target 'Neurophysiology and CSF Evaluation' (from 'Axonal Degeneration and Conduction Failure') not found in named elements
Pathograph: causal mechanism network for Acute Motor and Sensory Axonal Neuropathy Interactive directed graph showing how pathophysiology mechanisms, phenotypes, genetic factors and variants, experimental models, environmental triggers, and treatments relate through causal and linked edges.
●

Phenotypes

7
Digestive 1
Bulbar Dysfunction Dysphagia (HP:0002015)
Show evidence (1 reference)
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"urinary retention, dysarthria, dysphagia, bilateral facial paralysis, facial diplegia, bucking, and motor alalia gradually appeared"
The case report supports bulbar and cranial involvement during clinical worsening in AMSAN.
Limbs 1
Limb Weakness Limb muscle weakness (HP:0003690)
Course: PROGRESSIVE
Show evidence (1 reference)
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"presented to the emergency room with complaints of weakness and paraesthesia in both her legs and arms for 4 days"
This AMSAN case report directly documents acute limb weakness affecting both upper and lower limbs.
Nervous System 3
Areflexia Areflexia (HP:0001284)
Show evidence (1 reference)
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"Neurological examination disclosed symmetric weakness (Medical Research Council grade upper limbs 4/5, lowers limbs 2/5) and areflexia in both the legs and feet."
The case report documents areflexia in the lower limbs during AMSAN presentation.
Sensory Neuropathy Sensory neuropathy (HP:0000763)
Show evidence (1 reference)
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"The AMSAN variant of GBS secondary to COVID-19 infection is severe and can cause extensive damage to the peripheral nerves system."
This AMSAN-specific conclusion supports peripheral sensory-motor nerve involvement as part of the syndrome.
Paresthesia Paresthesia (HP:0003401)
Show evidence (1 reference)
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"presented to the emergency room with complaints of weakness and paraesthesia in both her legs and arms for 4 days"
The AMSAN case report explicitly documents paraesthesia accompanying limb weakness.
Respiratory 1
Respiratory Insufficiency Respiratory insufficiency (HP:0002093)
Show evidence (1 reference)
DOI:10.1007/s12028-023-01707-3 PARTIAL Human Clinical
"Of adult patients with GBS, 10–30% require mechanical ventilation during the acute phase of the disease."
This GBS-wide guideline supports respiratory insufficiency as a severe acute complication relevant to AMSAN as a GBS subtype; it does not provide AMSAN-specific frequency.
Constitutional 1
Pain Pain (HP:0012531)
Show evidence (1 reference)
PMID:39687605 PARTIAL Human Clinical
"Pain is a serious manifestation in both the acute and chronic stages of Guillain-Barre syndrome (GBS)."
This prospective GBS cohort supports pain as a GBS-wide manifestation. It is marked PARTIAL because the evidence is not AMSAN-specific, although the same abstract reports association with axonal GBS.
🧬

Genetic Associations

1
No established monogenic etiology (Unknown)
💊

Treatments

4
Intravenous Immunoglobulin
Action: intravenous immunoglobulin therapy Ontology label: Pharmacotherapy NCIT:C15986
Agent: human immunoglobulin G ↗
IVIG is standard immunotherapy for GBS and is used in AMSAN cases, although some severe cases may fluctuate or require additional supportive therapy.
Target Phenotypes: Limb muscle weakness ↗
Show evidence (2 references)
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"The patient was subsequently treated with intravenous immune globulin (IVIG), which improved her neurological symptoms"
This AMSAN case report documents neurologic improvement after IVIG.
DOI:10.1111/jns.12646 SUPPORT Human Clinical
"Current treatments for GBS include intravenous immunoglobulin (IVIg) and plasma exchange, which may not sufficiently benefit severely affected patients."
This phase 3 GBS trial abstract identifies IVIG as current standard treatment for GBS; AMSAN is a GBS subtype.
Plasma Exchange
Action: plasma exchange Ontology label: Plasmapheresis NCIT:C15304
Plasma exchange is an established GBS therapy and may be used when symptoms are severe or fluctuate after initial treatment.
Target Phenotypes: Limb muscle weakness ↗
Show evidence (2 references)
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"After being hospitalized for 16 days, the patient underwent continuous plasma exchange (PE) treatment for a duration of 3 days."
The AMSAN case report documents plasma exchange after clinical worsening.
DOI:10.1111/jns.12646 SUPPORT Human Clinical
"Current treatments for GBS include intravenous immunoglobulin (IVIg) and plasma exchange, which may not sufficiently benefit severely affected patients."
This phase 3 trial abstract identifies plasma exchange as current standard treatment for GBS.
Mechanical Ventilation
Action: mechanical ventilation Ontology label: artificial respiration MAXO:0000503
Severe acute GBS can require ventilatory support; this is included as supportive care for respiratory insufficiency rather than disease-modifying immunotherapy.
Target Phenotypes: Respiratory insufficiency ↗
Show evidence (1 reference)
DOI:10.1007/s12028-023-01707-3 PARTIAL Human Clinical
"Of adult patients with GBS, 10–30% require mechanical ventilation during the acute phase of the disease."
This guideline supports mechanical ventilation as acute supportive care in severe GBS, relevant to AMSAN as a severe axonal subtype.
Repeat IVIG or IVIG-PLEX Combination Therapy
Action: Pharmacotherapy NCIT:C15986
Repeat IVIG dosing or plasma exchange followed by IVIG should not be assumed beneficial for GBS non-responders.
Show evidence (1 reference)
DOI:10.1002/mus.28265 REFUTE Human Clinical
"Randomized controlled trials show that repeat intravenous immunoglobulin (IVIG) dosing and plasma exchange (PLEX) followed by IVIG (combination therapy) have no additional therapeutic benefit in Guillain‐Barre Syndrome (GBS) non‐responders."
This refutes routine escalation to repeat IVIG or sequential PLEX plus IVIG solely because of initial non-response.
🌍

Environmental Factors

2
Campylobacter jejuni infection
Antecedent Campylobacter jejuni infection is a major trigger for axonal GBS through ganglioside molecular mimicry.
Show evidence (1 reference)
DOI:10.3390/microorganisms10112139 SUPPORT Human Clinical
"Preceding infection with Campylobacter jejuni (Cj) occurs in approximately 30% of patients with Guillain–Barre syndrome (GBS), and the risk of GBS following Cj infection is increased by 77 to 100-fold."
This directly supports Campylobacter jejuni infection as an antecedent risk factor for GBS, particularly axonal GBS.
SARS-CoV-2 infection
SARS-CoV-2 infection has been reported before AMSAN onset in individual case literature; this is case-level evidence rather than established population risk.
Show evidence (1 reference)
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"Acute motor-sensory axonal polyneuropathy (AMSAN), is a GBS variant associated with COVID-19."
This case report supports COVID-19-associated AMSAN as a reported trigger context.
🔬

Biochemical Markers

2
Elevated CSF Protein (Elevated)
Context: Cerebrospinal fluid protein elevation supporting GBS diagnosis
Show evidence (1 reference)
DOI:10.3389/fneur.2023.1227505 SUPPORT Human Clinical
"Cerebrospinal fluid (CSF) analysis showed elevated protein levels that confirmed the diagnosis of GBS."
The AMSAN case report documents elevated CSF protein in the diagnostic workup.
Anti-Ganglioside Antibodies (Associated)
Context: Antibodies to gangliosides are diagnostic and mechanistic markers in GBS subtypes and variants
Show evidence (1 reference)
PMID:37090048 SUPPORT Human Clinical
"Anti-gangliosides antibodies are diagnostic markers of GBS"
This supports anti-ganglioside antibodies as a subtype marker relevant to axonal GBS variants, including AMSAN.
🔬

Clinical Trials

1
NCT04752566 PHASE_III COMPLETED
Phase 3 multicenter, double-blind, randomized, placebo-controlled trial of eculizumab add-on therapy to IVIG in severe Guillain-Barre syndrome; the primary endpoint was not achieved.
Target Phenotypes: Limb muscle weakness ↗
Show evidence (2 references)
clinicaltrials:NCT04752566 SUPPORT Human Clinical
"This is a Phase 3, prospective, multicenter, placebo controlled, double blind, randomized study to investigate the efficacy and safety of eculizumab in participants with severe GBS"
The ClinicalTrials.gov summary supports the existence and design of the phase 3 eculizumab trial in severe GBS.
DOI:10.1111/jns.12646 PARTIAL Human Clinical
"Primary endpoint was not achieved (hazard ratio, 0.9; 95% CI, 0.45–1.97; p = .89)."
The published phase 3 trial is relevant to complement-directed treatment in severe GBS, but the primary efficacy endpoint was not met.
{ }

Source YAML

click to show 
name: Acute Motor and Sensory Axonal Neuropathy
creation_date: '2026-05-07T23:29:25Z'
updated_date: '2026-05-08T00:03:01Z'
category: Autoimmune
parents:
- Guillain-Barre Syndrome
- Peripheral Neuropathy
disease_term:
  preferred_term: Acute motor and sensory axonal neuropathy
  term:
    id: MONDO:0020348
    label: acute motor and sensory axonal neuropathy
description: >-
  Acute motor and sensory axonal neuropathy (AMSAN) is an acute immune-mediated
  axonal variant of Guillain-Barre syndrome with motor and sensory peripheral
  nerve involvement. It is typically considered a post-infectious autoimmune
  neuropathy in which anti-ganglioside antibodies and complement-mediated nodal
  or axonal injury contribute to rapidly progressive weakness, sensory
  symptoms, and areflexia.
synonyms:
- Acute motor-sensory axonal polyneuropathy
- AMSAN
- Acute motor-sensory axonal Guillain-Barre syndrome
pathophysiology:
- name: Post-Infectious Molecular Mimicry
  description: >-
    Campylobacter jejuni infection can trigger antibodies that cross-react with
    peripheral nerve gangliosides through molecular mimicry, providing an
    upstream immune mechanism for axonal GBS variants including AMSAN.
  biological_processes:
  - preferred_term: Adaptive immune response
    term:
      id: GO:0002250
      label: adaptive immune response
  - preferred_term: Immunoglobulin production
    term:
      id: GO:0002377
      label: immunoglobulin production
  cell_types:
  - preferred_term: B cell lineage lymphocyte
    term:
      id: CL:0000945
      label: lymphocyte of B lineage
  evidence:
  - reference: DOI:10.3390/microorganisms10112139
    reference_title: "Campylobacter jejuni Infection, Anti-Ganglioside Antibodies, and Neuropathy"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      GBS is most often of the axonal subtype and is thought to be mediated by
      IgG antibodies to peripheral nerve gangliosides that are cross reactive
      with oligosaccharides in the Cj lipopolysaccharides (LPS).
    explanation: >-
      This review links axonal GBS to anti-ganglioside IgG induced by
      Campylobacter jejuni molecular mimicry, supporting the upstream immune
      mechanism applied to AMSAN as an axonal GBS variant.
  - reference: DOI:10.3390/microorganisms10112139
    reference_title: "Campylobacter jejuni Infection, Anti-Ganglioside Antibodies, and Neuropathy"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      The antibodies are thought to be induced by molecular mimicry, where
      immune reactivity to a cross reactive epitope in the infectious organism
      and normal tissue can cause autoimmune disease.
    explanation: >-
      The abstract explicitly states the molecular-mimicry model that explains
      how an antecedent infection can produce cross-reactive autoimmunity.
  downstream:
  - target: Ganglioside Antibody Binding at Nodes of Ranvier
    causal_link_type: INDIRECT_KNOWN_INTERMEDIATES
    intermediate_mechanisms:
    - Cross-reactive anti-ganglioside IgG production
    description: >-
      Infection-associated molecular mimicry produces anti-ganglioside antibodies
      that can bind peripheral nerve ganglioside targets.
- name: Ganglioside Antibody Binding at Nodes of Ranvier
  description: >-
    Anti-ganglioside antibodies bind ganglioside targets on peripheral nerve
    fibers, especially nodal and axolemmal regions implicated in axonal GBS
    variants.
  biological_processes:
  - preferred_term: Immune response
    term:
      id: GO:0006955
      label: immune response
  cell_types:
  - preferred_term: Neuron
    term:
      id: CL:0000540
      label: neuron
  evidence:
  - reference: PMID:37090048
    reference_title: "Detection of anti-ganglioside antibodies in Guillain-Barré syndrome."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      gangliosides can activate complement system and recruit macrophages on the
    explanation: >-
      This supports antibody binding to gangliosides as the upstream interaction
      in ganglioside-antibody mediated GBS.
  - reference: PMID:22507308
    reference_title: "A common mechanism and a new categorization for anti-ganglioside antibody-mediated neuropathies."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Serum antibodies to different gangliosides have been identified in some
    explanation: >-
      The review supports antiganglioside antibodies as a shared immune feature
      across GBS subtypes and variants, including axonal variants.
  downstream:
  - target: Complement Activation at Axolemma
    causal_link_type: DIRECT
    description: >-
      Ganglioside-bound antibodies can activate complement on peripheral nerve
      membranes.
- name: Complement Activation at Axolemma
  description: >-
    Complement-fixing anti-ganglioside antibodies activate complement at
    peripheral nerve axolemmal and nodal regions. Complement activation is
    modeled as a distinct effector step upstream of immune-cell recruitment and
    axonal injury.
  biological_processes:
  - preferred_term: Complement activation
    term:
      id: GO:0006956
      label: complement activation
  cell_types:
  - preferred_term: Neuron
    term:
      id: CL:0000540
      label: neuron
  evidence:
  - reference: PMID:37090048
    reference_title: "Detection of anti-ganglioside antibodies in Guillain-Barré syndrome."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      gangliosides can activate complement system and recruit macrophages on the
    explanation: >-
      This supports complement activation as a consequence of anti-ganglioside
      antibody binding in GBS.
  - reference: PMID:26936605
    reference_title: "C1q-targeted inhibition of the classical complement pathway prevents injury in a novel mouse model of acute motor axonal neuropathy."
    supports: PARTIAL
    evidence_source: MODEL_ORGANISM
    snippet: >-
      attenuated complement cascade activation and deposition, reduced immune cell
    explanation: >-
      This animal-model evidence supports complement activation and deposition
      as a mechanistic step in acute axonal GBS models; it is relevant to AMSAN
      mechanism but is not direct human AMSAN evidence.
  downstream:
  - target: Macrophage Recruitment to Complement Deposition Sites
    causal_link_type: DIRECT
    description: >-
      Complement activation and deposition recruit immune cells to affected
      peripheral nerve sites.
  - target: Axonal Degeneration and Conduction Failure
    causal_link_type: INDIRECT_KNOWN_INTERMEDIATES
    intermediate_mechanisms:
    - Complement deposition
    - Immune-cell recruitment
    description: >-
      Complement deposition is upstream of immune-cell recruitment and axonal
      injury in the acute axonal GBS model.
- name: Macrophage Recruitment to Complement Deposition Sites
  description: >-
    Complement deposition at peripheral nerve membranes recruits macrophages and
    other immune cells to the injury site; this node captures recruitment rather
    than antibody binding or axonal degeneration.
  biological_processes:
  - preferred_term: Macrophage chemotaxis
    term:
      id: GO:0048246
      label: macrophage chemotaxis
  cell_types:
  - preferred_term: Macrophage
    term:
      id: CL:0000235
      label: macrophage
  evidence:
  - reference: PMID:37090048
    reference_title: "Detection of anti-ganglioside antibodies in Guillain-Barré syndrome."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      gangliosides can activate complement system and recruit macrophages on the
    explanation: >-
      This supports macrophage recruitment downstream of ganglioside-antibody
      interactions in GBS.
  - reference: PMID:26936605
    reference_title: "C1q-targeted inhibition of the classical complement pathway prevents injury in a novel mouse model of acute motor axonal neuropathy."
    supports: PARTIAL
    evidence_source: MODEL_ORGANISM
    snippet: >-
      attenuated complement cascade activation and deposition, reduced immune cell
    explanation: >-
      This model-organism evidence links complement inhibition to reduced immune
      cell recruitment in an acute axonal GBS model.
  downstream:
  - target: Axonal Degeneration and Conduction Failure
    causal_link_type: DIRECT
    description: >-
      Recruited immune cells contribute to peripheral nerve axonal injury.
- name: Axonal Degeneration and Conduction Failure
  description: >-
    Axonal injury in AMSAN produces sensory-motor peripheral nerve dysfunction,
    including reduced conduction, progressive limb weakness, sensory symptoms,
    and neurophysiologic evidence of an acute motor-sensory axonal GBS pattern.
  cell_types:
  - preferred_term: Neuron
    term:
      id: CL:0000540
      label: neuron
  evidence:
  - reference: PMID:26936605
    reference_title: "C1q-targeted inhibition of the classical complement pathway prevents injury in a novel mouse model of acute motor axonal neuropathy."
    supports: PARTIAL
    evidence_source: MODEL_ORGANISM
    snippet: >-
      attenuated complement cascade activation and deposition, reduced immune cell
    explanation: >-
      This animal-model evidence supports axonal injury as a downstream
      consequence of complement-mediated acute axonal GBS mechanisms.
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Following a neuro-electro-physiology study to evaluate neurological
      symptoms, AMSAN was suggested.
    explanation: >-
      This AMSAN case report supports neurophysiologic evidence of the downstream
      acute motor-sensory axonal neuropathy state.
  downstream:
  - target: Limb Weakness
    causal_link_type: INDIRECT_KNOWN_INTERMEDIATES
    intermediate_mechanisms:
    - Peripheral motor axon dysfunction
    description: >-
      Motor axonal dysfunction causes progressive limb weakness.
  - target: Sensory Neuropathy
    causal_link_type: INDIRECT_KNOWN_INTERMEDIATES
    intermediate_mechanisms:
    - Peripheral sensory axon dysfunction
    description: >-
      Sensory axonal dysfunction causes sensory neuropathy and paresthesia.
  - target: Neurophysiology and CSF Evaluation
    causal_link_type: INDIRECT_KNOWN_INTERMEDIATES
    intermediate_mechanisms:
    - Acute motor-sensory axonal neuropathy pattern
    description: >-
      The downstream axonal injury state is detected by neurophysiologic testing
      used in AMSAN diagnosis.
phenotypes:
- name: Limb Weakness
  category: Neurological
  phenotype_term:
    preferred_term: Limb muscle weakness
    term:
      id: HP:0003690
      label: Limb muscle weakness
    clinical_course: PROGRESSIVE
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      presented to the emergency room with complaints of weakness and
      paraesthesia in both her legs and arms for 4 days
    explanation: >-
      This AMSAN case report directly documents acute limb weakness affecting
      both upper and lower limbs.
- name: Areflexia
  category: Neurological
  phenotype_term:
    preferred_term: Areflexia
    term:
      id: HP:0001284
      label: Areflexia
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Neurological examination disclosed symmetric weakness (Medical Research
      Council grade upper limbs 4/5, lowers limbs 2/5) and areflexia in both the
      legs and feet.
    explanation: >-
      The case report documents areflexia in the lower limbs during AMSAN
      presentation.
- name: Sensory Neuropathy
  category: Neurological
  phenotype_term:
    preferred_term: Sensory neuropathy
    term:
      id: HP:0000763
      label: Sensory neuropathy
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      The AMSAN variant of GBS secondary to COVID-19 infection is severe and can
      cause extensive damage to the peripheral nerves system.
    explanation: >-
      This AMSAN-specific conclusion supports peripheral sensory-motor nerve
      involvement as part of the syndrome.
- name: Paresthesia
  category: Neurological
  phenotype_term:
    preferred_term: Paresthesia
    term:
      id: HP:0003401
      label: Paresthesia
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      presented to the emergency room with complaints of weakness and
      paraesthesia in both her legs and arms for 4 days
    explanation: >-
      The AMSAN case report explicitly documents paraesthesia accompanying limb
      weakness.
- name: Pain
  category: Neurological
  phenotype_term:
    preferred_term: Pain
    term:
      id: HP:0012531
      label: Pain
  evidence:
  - reference: PMID:39687605
    reference_title: "Pain determinants and quality of life in Guillain-Barre syndrome: a prospective cohort study."
    supports: PARTIAL
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Pain is a serious manifestation in both the acute and chronic stages
      of Guillain-Barre syndrome (GBS).
    explanation: >-
      This prospective GBS cohort supports pain as a GBS-wide manifestation. It
      is marked PARTIAL because the evidence is not AMSAN-specific, although the
      same abstract reports association with axonal GBS.
- name: Bulbar Dysfunction
  category: Neurological
  phenotype_term:
    preferred_term: Dysphagia
    term:
      id: HP:0002015
      label: Dysphagia
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      urinary retention, dysarthria, dysphagia, bilateral facial paralysis,
      facial diplegia, bucking, and motor alalia gradually appeared
    explanation: >-
      The case report supports bulbar and cranial involvement during clinical
      worsening in AMSAN.
- name: Respiratory Insufficiency
  category: Respiratory
  phenotype_term:
    preferred_term: Respiratory insufficiency
    term:
      id: HP:0002093
      label: Respiratory insufficiency
  evidence:
  - reference: DOI:10.1007/s12028-023-01707-3
    reference_title: "Guidelines for Neuroprognostication in Adults with Guillain–Barré Syndrome"
    supports: PARTIAL
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Of adult patients with GBS, 10–30% require mechanical ventilation during
      the acute phase of the disease.
    explanation: >-
      This GBS-wide guideline supports respiratory insufficiency as a severe
      acute complication relevant to AMSAN as a GBS subtype; it does not provide
      AMSAN-specific frequency.
biochemical:
- name: Elevated CSF Protein
  presence: Elevated
  context: Cerebrospinal fluid protein elevation supporting GBS diagnosis
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Cerebrospinal fluid (CSF) analysis showed elevated protein levels that
      confirmed the diagnosis of GBS.
    explanation: >-
      The AMSAN case report documents elevated CSF protein in the diagnostic
      workup.
- name: Anti-Ganglioside Antibodies
  presence: Associated
  context: Antibodies to gangliosides are diagnostic and mechanistic markers in GBS subtypes and variants
  evidence:
  - reference: PMID:37090048
    reference_title: "Detection of anti-ganglioside antibodies in Guillain-Barré syndrome."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Anti-gangliosides antibodies are diagnostic markers of GBS
    explanation: >-
      This supports anti-ganglioside antibodies as a subtype marker relevant to
      axonal GBS variants, including AMSAN.
genetic:
- name: No established monogenic etiology
  association: Unknown
  notes: >-
    AMSAN is treated here as an acquired immune-mediated GBS variant. No
    AMSAN-specific causal gene or pathogenic DNA variant was identified in the
    Falcon report, so no gene binding is asserted.
environmental:
- name: Campylobacter jejuni infection
  presence: Risk factor
  description: >-
    Antecedent Campylobacter jejuni infection is a major trigger for axonal GBS
    through ganglioside molecular mimicry.
  evidence:
  - reference: DOI:10.3390/microorganisms10112139
    reference_title: "Campylobacter jejuni Infection, Anti-Ganglioside Antibodies, and Neuropathy"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Preceding infection with Campylobacter jejuni (Cj) occurs in approximately
      30% of patients with Guillain–Barre syndrome (GBS), and the risk of GBS
      following Cj infection is increased by 77 to 100-fold.
    explanation: >-
      This directly supports Campylobacter jejuni infection as an antecedent risk
      factor for GBS, particularly axonal GBS.
- name: SARS-CoV-2 infection
  presence: Reported trigger
  description: >-
    SARS-CoV-2 infection has been reported before AMSAN onset in individual case
    literature; this is case-level evidence rather than established population
    risk.
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Acute motor-sensory axonal polyneuropathy (AMSAN), is a GBS variant
      associated with COVID-19.
    explanation: >-
      This case report supports COVID-19-associated AMSAN as a reported trigger
      context.
diagnosis:
- name: Neurophysiology and CSF Evaluation
  description: >-
    AMSAN diagnosis is supported by neurophysiology showing an acute motor-sensory
    axonal GBS pattern and by CSF protein elevation.
  diagnosis_term:
    preferred_term: diagnostic procedure
    term:
      id: MAXO:0000003
      label: diagnostic procedure
  results: Neurophysiology suggests AMSAN; elevated CSF protein supports GBS.
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Following a neuro-electro-physiology study to evaluate neurological
      symptoms, AMSAN was suggested. Cerebrospinal fluid (CSF) analysis showed
      elevated protein levels that confirmed the diagnosis of GBS.
    explanation: >-
      The case report directly links neuro-electrophysiology and CSF protein
      elevation to AMSAN/GBS diagnosis.
- name: Anti-Ganglioside Antibody Testing
  description: >-
    Serologic testing for anti-ganglioside autoantibodies can support diagnosis
    and subtype classification in autoimmune peripheral neuropathies.
  diagnosis_term:
    preferred_term: serology testing
    term:
      id: MAXO:0000609
      label: serology testing
  evidence:
  - reference: PMID:37090048
    reference_title: "Detection of anti-ganglioside antibodies in Guillain-Barré syndrome."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Testing of anti-gangliosides autoantibodies is helpful for
    explanation: >-
      This supports anti-ganglioside antibody testing as a diagnostic adjunct for
      autoimmune peripheral neuropathy subtype classification.
treatments:
- name: Intravenous Immunoglobulin
  description: >-
    IVIG is standard immunotherapy for GBS and is used in AMSAN cases, although
    some severe cases may fluctuate or require additional supportive therapy.
  treatment_term:
    preferred_term: intravenous immunoglobulin therapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
    therapeutic_agent:
    - preferred_term: human immunoglobulin G
      term:
        id: NCIT:C80829
        label: Human Immunoglobulin G
  target_phenotypes:
  - preferred_term: Limb muscle weakness
    term:
      id: HP:0003690
      label: Limb muscle weakness
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      The patient was subsequently treated with intravenous immune globulin
      (IVIG), which improved her neurological symptoms
    explanation: >-
      This AMSAN case report documents neurologic improvement after IVIG.
  - reference: DOI:10.1111/jns.12646
    reference_title: "Efficacy and safety of eculizumab in Guillain‐Barré syndrome: A phase 3, multicenter, double‐blind, randomized, placebo‐controlled clinical trial"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Current treatments for GBS include intravenous immunoglobulin (IVIg) and
      plasma exchange, which may not sufficiently benefit severely affected
      patients.
    explanation: >-
      This phase 3 GBS trial abstract identifies IVIG as current standard
      treatment for GBS; AMSAN is a GBS subtype.
  notes: >-
    OAK verification on 2026-05-08 showed that MAXO:0000412 is "biopsy of
    tongue" in the local MAXO adapter, not intravenous immunoglobulin therapy;
    the generic pharmacotherapy action term is therefore retained with
    therapeutic_agent carrying the immunoglobulin identity.
- name: Plasma Exchange
  description: >-
    Plasma exchange is an established GBS therapy and may be used when symptoms
    are severe or fluctuate after initial treatment.
  treatment_term:
    preferred_term: plasma exchange
    term:
      id: NCIT:C15304
      label: Plasmapheresis
  target_phenotypes:
  - preferred_term: Limb muscle weakness
    term:
      id: HP:0003690
      label: Limb muscle weakness
  evidence:
  - reference: DOI:10.3389/fneur.2023.1227505
    reference_title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      After being hospitalized for 16 days, the patient underwent continuous
      plasma exchange (PE) treatment for a duration of 3 days.
    explanation: >-
      The AMSAN case report documents plasma exchange after clinical worsening.
  - reference: DOI:10.1111/jns.12646
    reference_title: "Efficacy and safety of eculizumab in Guillain‐Barré syndrome: A phase 3, multicenter, double‐blind, randomized, placebo‐controlled clinical trial"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Current treatments for GBS include intravenous immunoglobulin (IVIg) and
      plasma exchange, which may not sufficiently benefit severely affected
      patients.
    explanation: >-
      This phase 3 trial abstract identifies plasma exchange as current standard
      treatment for GBS.
- name: Mechanical Ventilation
  description: >-
    Severe acute GBS can require ventilatory support; this is included as
    supportive care for respiratory insufficiency rather than disease-modifying
    immunotherapy.
  treatment_term:
    preferred_term: mechanical ventilation
    term:
      id: MAXO:0000503
      label: artificial respiration
  target_phenotypes:
  - preferred_term: Respiratory insufficiency
    term:
      id: HP:0002093
      label: Respiratory insufficiency
  evidence:
  - reference: DOI:10.1007/s12028-023-01707-3
    reference_title: "Guidelines for Neuroprognostication in Adults with Guillain–Barré Syndrome"
    supports: PARTIAL
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Of adult patients with GBS, 10–30% require mechanical ventilation during
      the acute phase of the disease.
    explanation: >-
      This guideline supports mechanical ventilation as acute supportive care in
      severe GBS, relevant to AMSAN as a severe axonal subtype.
- name: Repeat IVIG or IVIG-PLEX Combination Therapy
  description: >-
    Repeat IVIG dosing or plasma exchange followed by IVIG should not be assumed
    beneficial for GBS non-responders.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
  evidence:
  - reference: DOI:10.1002/mus.28265
    reference_title: "Intravenous immunoglobulin and plasma exchange prescribing patterns for Guillain‐Barre Syndrome in the United States—2001 to 2018"
    supports: REFUTE
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Randomized controlled trials show that repeat intravenous immunoglobulin
      (IVIG) dosing and plasma exchange (PLEX) followed by IVIG (combination
      therapy) have no additional therapeutic benefit in Guillain‐Barre Syndrome
      (GBS) non‐responders.
    explanation: >-
      This refutes routine escalation to repeat IVIG or sequential PLEX plus IVIG
      solely because of initial non-response.
clinical_trials:
- name: NCT04752566
  phase: PHASE_III
  status: COMPLETED
  description: >-
    Phase 3 multicenter, double-blind, randomized, placebo-controlled trial of
    eculizumab add-on therapy to IVIG in severe Guillain-Barre syndrome; the
    primary endpoint was not achieved.
  target_phenotypes:
  - preferred_term: Limb muscle weakness
    term:
      id: HP:0003690
      label: Limb muscle weakness
  evidence:
  - reference: clinicaltrials:NCT04752566
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      This is a Phase 3, prospective, multicenter, placebo controlled, double
      blind, randomized study to investigate the efficacy and safety of
      eculizumab in participants with severe GBS
    explanation: >-
      The ClinicalTrials.gov summary supports the existence and design of the
      phase 3 eculizumab trial in severe GBS.
  - reference: DOI:10.1111/jns.12646
    reference_title: "Efficacy and safety of eculizumab in Guillain‐Barré syndrome: A phase 3, multicenter, double‐blind, randomized, placebo‐controlled clinical trial"
    supports: PARTIAL
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Primary endpoint was not achieved (hazard ratio, 0.9; 95% CI,
      0.45–1.97; p = .89).
    explanation: >-
      The published phase 3 trial is relevant to complement-directed treatment
      in severe GBS, but the primary efficacy endpoint was not met.
  notes: >-
    Included as GBS-wide interventional evidence relevant to AMSAN as an axonal
    GBS variant; the trial did not report AMSAN-specific efficacy.
references:
- reference: DOI:10.3389/fneur.2023.1227505
  title: "Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism"
  findings: []
- reference: DOI:10.3390/microorganisms10112139
  title: "Campylobacter jejuni Infection, Anti-Ganglioside Antibodies, and Neuropathy"
  findings: []
- reference: PMID:37090048
  title: "Detection of anti-ganglioside antibodies in Guillain-Barré syndrome."
  findings: []
- reference: PMID:22507308
  title: "A common mechanism and a new categorization for anti-ganglioside antibody-mediated neuropathies."
  findings: []
- reference: PMID:26936605
  title: "C1q-targeted inhibition of the classical complement pathway prevents injury in a novel mouse model of acute motor axonal neuropathy."
  findings: []
- reference: DOI:10.1111/jns.12646
  title: "Efficacy and safety of eculizumab in Guillain‐Barré syndrome: A phase 3, multicenter, double‐blind, randomized, placebo‐controlled clinical trial"
  findings: []
- reference: DOI:10.1007/s12028-023-01707-3
  title: "Guidelines for Neuroprognostication in Adults with Guillain–Barré Syndrome"
  findings: []
- reference: DOI:10.1002/mus.28265
  title: Intravenous immunoglobulin and plasma exchange prescribing patterns for Guillain‐Barre Syndrome in the United States—2001 to 2018
  findings: []
- reference: PMID:39687605
  title: "Pain determinants and quality of life in Guillain-Barre syndrome: a prospective cohort study."
  findings: []
- reference: clinicaltrials:NCT04752566
  title: "A Phase 3, Prospective, Multicenter, Double Blind, Randomized, Placebo Controlled Study to Evaluate the Efficacy and Safety of Eculizumab in Patients With Guillain-Barré Syndrome (GBS)"
  findings: []
📚

References & Deep Research

References

10
DOI:10.3389/fneur.2023.1227505
Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism
No top-level findings curated for this source.
DOI:10.3390/microorganisms10112139
Campylobacter jejuni Infection, Anti-Ganglioside Antibodies, and Neuropathy
No top-level findings curated for this source.
PMID:37090048
Detection of anti-ganglioside antibodies in Guillain-Barré syndrome.
No top-level findings curated for this source.
PMID:22507308
A common mechanism and a new categorization for anti-ganglioside antibody-mediated neuropathies.
No top-level findings curated for this source.
PMID:26936605
C1q-targeted inhibition of the classical complement pathway prevents injury in a novel mouse model of acute motor axonal neuropathy.
No top-level findings curated for this source.
DOI:10.1111/jns.12646
Efficacy and safety of eculizumab in Guillain‐Barré syndrome: A phase 3, multicenter, double‐blind, randomized, placebo‐controlled clinical trial
No top-level findings curated for this source.
DOI:10.1007/s12028-023-01707-3
Guidelines for Neuroprognostication in Adults with Guillain–Barré Syndrome
No top-level findings curated for this source.
DOI:10.1002/mus.28265
Intravenous immunoglobulin and plasma exchange prescribing patterns for Guillain‐Barre Syndrome in the United States—2001 to 2018
No top-level findings curated for this source.
PMID:39687605
Pain determinants and quality of life in Guillain-Barre syndrome: a prospective cohort study.
No top-level findings curated for this source.
clinicaltrials:NCT04752566
A Phase 3, Prospective, Multicenter, Double Blind, Randomized, Placebo Controlled Study to Evaluate the Efficacy and Safety of Eculizumab in Patients With Guillain-Barré Syndrome (GBS)
No top-level findings curated for this source.

Deep Research

1
Falcon ▸
Acute Motor and Sensory Axonal Neuropathy (AMSAN) — Comprehensive Disease Characteristics Report
Edison Scientific Literature 27 citations 2026-05-07T19:42:51.902600

Acute Motor and Sensory Axonal Neuropathy (AMSAN) — Comprehensive Disease Characteristics Report

Target disease: Acute motor and sensory axonal neuropathy (AMSAN) (autoimmune; acute post-infectious immune-mediated peripheral neuropathy; axonal Guillain–Barré syndrome (GBS) variant). (shang2021axonalvariantsof pages 1-2)

High-level definition (current understanding): AMSAN is an axonal form of GBS with acute involvement of motor and sensory axons, typically presenting with rapidly progressive limb weakness plus sensory loss/paresthesia and areflexia/hyporeflexia, often after an infectious trigger. (shang2021axonalvariantsof pages 1-2, leonhard2024guillain–barrésyndrome pages 1-3)

Key abstract-quoted definition (GBS umbrella): Nature Reviews Disease Primers (published 2024-12-19) states: “Guillain–Barré syndrome (GBS) is a rare immune-mediated polyradiculoneuropathy” and notes that “Diagnosis is based on clinical features, supported by cerebrospinal fluid analysis and nerve conduction studies.” (leonhard2024guillain–barrésyndrome pages 1-3)

1. Disease Information

1.1 Overview

AMSAN is a GBS subtype within “axonal variants” (primarily AMAN and AMSAN). AMSAN differs from AMAN by having prominent sensory axonal involvement in addition to motor axonal injury. (shang2021axonalvariantsof pages 1-2)

1.2 Key identifiers / ontology mappings (availability in retrieved sources)

The retrieved literature focused on GBS and axonal variants but did not provide a specific MONDO ID or Orphanet code for AMSAN.

ICD (from real-world claims-based definitions for GBS): - GBS was defined using ICD-9 357.0 and ICD-10 G61.0, G65.0 in a US prescribing-patterns study. (stino2024intravenousimmunoglobulinand pages 1-2)

MeSH / MONDO / Orphanet / OMIM: not explicitly stated in the retrieved texts; thus not reliably reportable here.

1.3 Synonyms / alternative names

  • “Acute motor-sensory axonal polyneuropathy” (used as a synonym in case literature). (geng2023acutemotorsensoryaxonal pages 3-5)
  • “Axonal Guillain–Barré syndrome” (umbrella term encompassing AMAN/AMSAN). (umar2024complexneurologicalsequelae pages 4-6, shang2021axonalvariantsof pages 1-2)

1.4 Evidence source type

Most disease-level statements here are derived from aggregated disease resources and reviews (e.g., Nature Reviews Disease Primers; axonal GBS update review; Campylobacter–ganglioside review), supplemented by human clinical case reports illustrating AMSAN phenotypes/triggers and treatment response. (leonhard2024guillain–barrésyndrome pages 1-3, shang2021axonalvariantsof pages 1-2, latov2022campylobacterjejuniinfection pages 1-2, geng2023acutemotorsensoryaxonal pages 3-5)

2. Etiology

2.1 Primary causal factors (mechanistic etiology)

Post-infectious autoimmunity via molecular mimicry is the leading paradigm for axonal GBS/AMSAN, particularly in Campylobacter jejuni–associated disease. (shang2021axonalvariantsof pages 1-2, latov2022campylobacterjejuniinfection pages 1-2, leonhard2024guillain–barrésyndrome pages 1-3)

Abstract quote (Campylobacter–GBS link; published 2022-10-28): Latov writes: “Preceding infection with Campylobacter jejuni (Cj) occurs in approximately 30% of patients with Guillain–Barre syndrome (GBS), and the risk of GBS following Cj infection is increased by 77 to 100-fold. GBS is most often of the axonal subtype and is thought to be mediated by IgG antibodies to peripheral nerve gangliosides … induced by molecular mimicry.” (latov2022campylobacterjejuniinfection pages 1-2)

2.2 Risk factors

Infectious triggers (human clinical + epidemiologic evidence): - Campylobacter jejuni enteritis: strong epidemiologic association and mechanistic evidence via ganglioside mimicry. (latov2022campylobacterjejuniinfection pages 1-2, leonhard2024guillain–barrésyndrome pages 1-3) - Viral and other infectious triggers reported for axonal variants include Zika and other pathogens in review summaries; case literature also reports post–COVID-19 AMSAN. (shang2021axonalvariantsof pages 1-2, geng2023acutemotorsensoryaxonal pages 3-5)

Timing after diarrheal illness: in C. jejuni–associated GBS, neurological symptoms “usually begin at 10 days to 3 weeks after the onset of diarrhea.” (latov2022campylobacterjejuniinfection pages 1-2)

COVID-19 association (case-level, mechanistic hypotheses): AMSAN has been described following SARS-CoV-2 infection, with proposed mechanisms including molecular mimicry and hyperinflammatory para-infectious immune injury; anti-ganglioside antibodies may be absent in some cases. (geng2023acutemotorsensoryaxonal pages 3-5, geng2023acutemotorsensoryaxonal pages 5-6)

2.3 Protective factors

No specific protective genetic variants, environmental protective factors, or proven preventive interventions for AMSAN were identified in the retrieved sources.

2.4 Gene–environment interactions

The retrieved sources emphasize infection-triggered autoimmunity and mention “host genetic predisposition” as an important research direction in GBS broadly, but do not provide validated AMSAN-specific gene–environment interaction loci. (shang2021axonalvariantsof pages 1-2)

3. Phenotypes (clinical features)

3.1 Core phenotype spectrum (suggested HPO terms)

AMSAN typically includes: - Acute/subacute limb weakness (often symmetric), progressing over days (HP:0001324 Muscle weakness; HP:0003674 Motor delay/impairment not specific; consider HP:0003323 Progressive muscular weakness). - Areflexia/hyporeflexia (HP:0001284 Areflexia). (leonhard2024guillain–barrésyndrome pages 1-3) - Sensory symptoms (paresthesia, sensory loss) (HP:0003401 Paresthesia; HP:0000763 Sensory neuropathy). (leonhard2024guillain–barrésyndrome pages 1-3, geng2023acutemotorsensoryaxonal pages 3-5) - Pain (HP:0012531 Pain). (leonhard2024guillain–barrésyndrome pages 1-3) - Cranial nerve involvement may occur in severe GBS phenotypes (facial palsy, bulbar weakness) (HP:0001343 Facial palsy; HP:0002493 Dysphagia). (leonhard2024guillain–barrésyndrome pages 1-3) - Autonomic dysfunction in severe cases (HP:0001278 Orthostatic hypotension; HP:0002013 Vomiting; broader: dysautonomia). (busl2023guidelinesforneuroprognostication pages 1-3, umar2024complexneurologicalsequelae pages 4-6) - Respiratory failure / need for ventilation in severe cases (HP:0002878 Respiratory failure; HP:0002094 Dyspnea). (leonhard2024guillain–barrésyndrome pages 1-3)

3.2 Onset and progression

GBS (including AMSAN) is characterized by rapid progression with a typical nadir within weeks. Neurocritical Care guidelines note symptoms reach maximum “within 2–4 weeks.” (busl2023guidelinesforneuroprognostication pages 1-3)

3.3 Frequency among affected individuals (data availability)

Phenotype frequencies are best described for GBS overall rather than AMSAN specifically in the retrieved sources: - “Around 20% of patients may develop weakness in all four limbs … and respiratory failure requiring mechanical ventilation.” (leonhard2024guillain–barrésyndrome pages 1-3)

3.4 Quality-of-life impact

Nature Reviews Disease Primers emphasizes residual disability: “~20% of patients who received treatment are unable to walk after 6 months.” (leonhard2024guillain–barrésyndrome pages 1-3)

4. Genetic/Molecular Information

4.1 Causal genes

AMSAN is not a monogenic disorder in standard clinical framing; the retrieved sources do not identify causal genes or OMIM disease entries specific to AMSAN. (leonhard2024guillain–barrésyndrome pages 1-3, shang2021axonalvariantsof pages 1-2)

4.2 Key molecular targets (autoantigens)

Gangliosides on peripheral nerves are key immune targets in axonal GBS/AMSAN: - Anti-ganglioside antibodies reported in association with AMSAN include anti-GM1, anti-GM1b, anti-GD1a. (shang2021axonalvariantsof pages 6-7)

4.3 Pathogenic “variants” (not applicable)

Pathogenic DNA variants are not established as causal for AMSAN in the provided evidence.

4.4 Epigenetics / chromosomal abnormalities

Not established for AMSAN in the retrieved sources.

5. Environmental Information

5.1 Infectious agents (key environmental triggers)

  • Campylobacter jejuni is the best-supported trigger for axonal GBS/AMSAN. (latov2022campylobacterjejuniinfection pages 1-2, leonhard2024guillain–barrésyndrome pages 1-3)
  • SARS-CoV-2 has been associated with AMSAN in case literature, with immune-mediated mechanisms proposed. (geng2023acutemotorsensoryaxonal pages 3-5, geng2023acutemotorsensoryaxonal pages 5-6)

5.2 Lifestyle/occupational factors

No AMSAN-specific lifestyle protective/risk factors were established in the retrieved sources.

6. Mechanism / Pathophysiology

6.1 Causal chain (upstream → downstream)

Upstream trigger: infection (especially C. jejuni; also viral triggers). (latov2022campylobacterjejuniinfection pages 1-2, leonhard2024guillain–barrésyndrome pages 1-3)

Immune priming via molecular mimicry: bacterial lipooligosaccharides mimic peripheral nerve gangliosides, producing cross-reactive antibodies. Nature Reviews Disease Primers explicitly summarizes: “For example, in patients with preceding Campylobacter jejuni infection, molecular mimicry causes a cross-reactive antibody response to nerve gangliosides.” (leonhard2024guillain–barrésyndrome pages 1-3)

Effector injury: anti-ganglioside antibodies activate complement at nodes/paranodes and axolemma, causing conduction failure and structural axonal injury/degeneration (axonal variants). (leonhard2024guillain–barrésyndrome pages 5-7, latov2022campylobacterjejuniinfection pages 2-4)

Downstream clinical manifestations: reduced compound muscle action potentials (CMAPs), sensory nerve action potential (SNAP) abnormalities, weakness + sensory loss, and in severe cases bulbar/respiratory/autonomic failure. (geng2023acutemotorsensoryaxonal pages 3-5, leonhard2024guillain–barrésyndrome pages 1-3)

6.2 Immune system involvement

  • Axonal variants are associated with anti-ganglioside antibodies and complement-dependent mechanisms; Latov describes that GBS axonal subtype is “thought to be mediated by IgG antibodies to peripheral nerve gangliosides” cross-reactive with C. jejuni components. (latov2022campylobacterjejuniinfection pages 1-2)

6.3 Suggested GO biological process terms (mechanism-oriented)

  • GO:0006956 complement activation
  • GO:0006955 immune response
  • GO:0002250 adaptive immune response
  • GO:0042113 B cell activation
  • GO:0007166 cell surface receptor signaling pathway (broad)

6.4 Suggested Cell Ontology terms (CL)

  • CL:0000945 B cell
  • CL:0000084 T cell
  • CL:0000235 macrophage
  • CL:0000584 Schwann cell (peripheral glia; mechanistic relevance to peripheral nerves broadly)

6.5 Molecular profiling / biomarkers (availability)

The retrieved sources highlight a lack of specific biomarkers for GBS broadly (including axonal variants). (leonhard2024guillain–barrésyndrome pages 1-3)

7. Anatomical Structures Affected

7.1 Organ/system level

  • Peripheral nervous system (peripheral nerves and roots; polyradiculoneuropathy). (leonhard2024guillain–barrésyndrome pages 1-3)

7.2 Tissue/cell level (suggested UBERON terms)

  • UBERON:0000010 peripheral nerve
  • UBERON:0001021 spinal nerve root

7.3 Subcellular (suggested GO Cellular Component)

  • GO:0033267 axon part
  • GO:0043209 myelin sheath (even in “axonal” variants, nerve architecture is involved)
  • GO:0030054 cell junction (node/paranode microdomains; mechanistic target region) (leonhard2024guillain–barrésyndrome pages 5-7)

8. Temporal Development

8.1 Onset pattern

Acute onset with rapid progression; nadir typically within 2–4 weeks in GBS overall. (busl2023guidelinesforneuroprognostication pages 1-3)

8.2 Course

Typically monophasic: Nature Reviews Disease Primers states “GBS is usually a monophasic disease.” (leonhard2024guillain–barrésyndrome pages 1-3)

9. Inheritance and Population

9.1 Epidemiology

Data are primarily for GBS overall; AMSAN-specific incidence is not provided in the retrieved evidence.

  • GBS annual global incidence “1–2 per 100,000 persons per year.” (leonhard2024guillain–barrésyndrome pages 1-3)
  • Axonal-variants review provides a similar range: 0.81–1.89 per 100,000/year. (shang2021axonalvariantsof pages 1-2)
  • Sex/age: males affected ~1.5× more than females; risk rises ~20% per decade of age. (leonhard2024guillain–barrésyndrome pages 1-3)

9.2 Population differences (axonal variants)

Axonal variants (including AMSAN) are discussed as having geographic variability, with higher representation in some regions (review-level). (restrepojimenez2018theimmunotherapyof pages 46-47)

10. Diagnostics

10.1 Diagnostic approach (real-world implementation)

GBS diagnosis is “based on clinical features, supported by cerebrospinal fluid analysis and nerve conduction studies.” (leonhard2024guillain–barrésyndrome pages 1-3)

CSF: - Axonal-variants review: CSF albuminocytologic dissociation is a hallmark “detectable in almost 90%,” with CSF albumin rising from week 2 and present in ~70% by the end of week 2. (shang2021axonalvariantsof pages 1-2)

Electrophysiology (NCS/EMG): - Axonal variants: early studies can be misleading; decreased CMAP amplitudes and reversible conduction failure/block can appear early; electrophysiology “more reliable” at 3–6 weeks than at 1–2 weeks. (shang2021axonalvariantsof pages 1-2) - Nature Reviews Disease Primers emphasizes heterogeneity: mixed axonal–demyelinating or even normal NCS can occur, limiting strict NCS-only subclassification. (leonhard2024guillain–barrésyndrome pages 4-5)

Serology (supportive, not required for all): Anti-ganglioside antibodies support axonal subtype classification (anti-GM1/anti-GD1a and related), but seronegative axonal cases exist. (shang2021axonalvariantsof pages 6-7, geng2023acutemotorsensoryaxonal pages 5-6)

10.2 Differential diagnosis (high-level)

  • Acute onset CIDP (A-CIDP) is clinically challenging and can lead to reclassification; this carries therapeutic implications. (stino2024intravenousimmunoglobulinand pages 1-2)

11. Outcome / Prognosis

11.1 Key outcome statistics (GBS overall)

Nature Reviews Disease Primers reports: “~20% of patients who received treatment are unable to walk after 6 months and ~5% die as a consequence of GBS.” (leonhard2024guillain–barrésyndrome pages 1-3)

Neurocritical Care neuroprognostication guidelines provide ICU-relevant statistics: - “10–30% require mechanical ventilation during the acute phase.” (busl2023guidelinesforneuroprognostication pages 1-3) - Mortality “range between 1 and 13%,” with “mortality rates up to 20%” among ventilated patients. (busl2023guidelinesforneuroprognostication pages 1-3)

11.2 Prognostic tools / expert consensus

Busl et al. recommend: - EGRIS (Erasmus GBS Respiratory Insufficiency Score) for predicting ventilation, and - EGOS / modified EGOS for predicting independent ambulation at 3 months and beyond. (busl2023guidelinesforneuroprognostication pages 1-3)

12. Treatment

12.1 Standard of care

Only proven effective disease-modifying treatments for GBS (including AMSAN) remain: - Intravenous immunoglobulin (IVIg) - Plasma exchange (PE/PLEX) (leonhard2024guillain–barrésyndrome pages 1-3)

Nature Reviews Disease Primers (2024) abstract quote: “Effective treatments include plasma exchange and intravenous immunoglobulins.” (leonhard2024guillain–barrésyndrome pages 1-3)

12.2 Treatment strategy and common pitfalls (guidelines + evidence)

A US real-world analysis emphasizes trials showing no benefit from: - Repeat IVIG dosing and - PLEX followed by IVIG (combination therapy) in non-responders. (stino2024intravenousimmunoglobulinand pages 1-2)

Abstract quote (real-world utilization; published 2024-09): Stino et al. state: “Randomized controlled trials show that repeat IVIG dosing and … combination therapy have no additional therapeutic benefit in Guillain-Barre Syndrome (GBS) non-responders.” (stino2024intravenousimmunoglobulinand pages 1-2)

12.3 Recent developments (2023–2024) — complement inhibition (eculizumab)

A key 2024 development is a phase 3 randomized trial of eculizumab (C5 inhibitor) added to IVIg in severe GBS.

Abstract quote (published 2024-07; J Peripher Nerv Syst): “This study evaluated the efficacy and safety of eculizumab add-on therapy to IVIg … in patients with severe GBS.” (kuwabara2024efficacyandsafety pages 1-2)

Results: - Primary endpoint not met (time to Hughes FG ≤1): HR 0.9, 95% CI 0.45–1.97; p = 0.89. (kuwabara2024efficacyandsafety pages 1-2) - Strong target engagement: serum free C5 reduced by 99.99% at 1 hour postdose and sustained to week 5. (kuwabara2024efficacyandsafety pages 1-2)

12.4 MAXO (Medical Action Ontology) suggestions

  • MAXO:0000412 intravenous immunoglobulin therapy
  • MAXO:0000756 therapeutic plasma exchange
  • MAXO:0000610 mechanical ventilation (for respiratory failure)
  • MAXO:0000571 physical therapy / rehabilitation (supportive)

13. Prevention

No established primary-prevention intervention is specific to AMSAN beyond prevention/management of infectious triggers at the population level.

Vaccine-associated GBS (broader context): The retrieved evidence supports ongoing pharmacovigilance and risk assessment for GBS after vaccination, but does not provide AMSAN-specific prevention guidance. (shang2021axonalvariantsof pages 1-2)

14. Other Species / Natural Disease

Direct naturally occurring AMSAN analogs in non-human species were not identified in the retrieved sources.

15. Model Organisms

Experimental autoimmune neuritis and anti-ganglioside models (mechanistic relevance): Latov reports animal models in which rabbits immunized with GM1 or C. jejuni LPS develop acute axonal neuropathy with anti-GM1 antibodies, supporting the antibody-mediated mechanism relevant to axonal GBS/AMSAN. (latov2022campylobacterjejuniinfection pages 2-4)

2023–2024 “latest research” highlights (prioritized)

  1. 2024 authoritative synthesis: Nature Reviews Disease Primers (Leonhard et al.; 2024-12-19) consolidates contemporary consensus on GBS triggers, diagnosis (CSF + NCS), treatment (IVIg/PE), and residual disability (20% non-ambulant at 6 months; ~5% mortality). (leonhard2024guillain–barrésyndrome pages 1-3)
  2. 2024 mechanism-directed therapy trial: Phase 3 add-on eculizumab to IVIg did not improve functional recovery despite robust complement suppression, suggesting complement blockade alone is insufficient or that patient selection/timing remain unresolved. (kuwabara2024efficacyandsafety pages 1-2)
  3. 2024 real-world implementation gap: A US claims-based cohort (2001–2018) found repeat IVIG use (39.7%) and combination therapy (6.1%) persisted, despite RCT evidence against these approaches in non-responders; diagnostic reclassification to CIDP occurred in 32%. (stino2024intravenousimmunoglobulinand pages 1-2)

Evidence summary table

Domain Evidence summary Key quantitative stats (with values) Primary source (first author, year, journal) PMID if available URL Context citation ID
Definition AMSAN is an axonal Guillain-Barré syndrome (GBS) subtype characterized by acute motor and sensory axonal involvement; axonal GBS variants include AMAN and AMSAN. GBS is an acute, immune-mediated polyradiculoneuropathy with rapidly progressive weakness and sensory deficits. Global GBS incidence: 1–2/100,000/year; males affected ~1.5× more often; risk rises ~20% per decade of age. Leonhard, 2024, Nature Reviews Disease Primers https://doi.org/10.1038/s41572-024-00580-4 (leonhard2024guillain–barrésyndrome pages 1-3, shang2021axonalvariantsof pages 1-2)
Triggers AMSAN is usually post-infectious, with Campylobacter jejuni the best-supported trigger; COVID-19, chikungunya, and other infections have also been reported as antecedents in axonal GBS/AMSAN cases. Molecular mimicry between microbial glycans and nerve gangliosides is the leading mechanism. Preceding C. jejuni in ~30% of GBS; GBS risk after C. jejuni infection increased 77–100-fold; neurologic symptoms usually begin 10 days to 3 weeks after diarrhea. Latov, 2022, Microorganisms https://doi.org/10.3390/microorganisms10112139 (latov2022campylobacterjejuniinfection pages 1-2, latov2022campylobacterjejuniinfection pages 2-4, geng2023acutemotorsensoryaxonal pages 3-5)
Autoantibodies Axonal GBS including AMSAN is associated most often with anti-ganglioside antibodies, particularly anti-GM1, anti-GM1b, and anti-GD1a. Antibodies are often IgG1/IgG3 subclasses and can cross-react with C. jejuni lipooligosaccharides. Anti-ganglioside antibodies reported in 41–85% of GBS following C. jejuni infection. Shang, 2021, Journal of Neurology https://doi.org/10.1007/s00415-020-09742-2 (shang2021axonalvariantsof pages 6-7, latov2022campylobacterjejuniinfection pages 2-4, latov2022campylobacterjejuniinfection pages 1-2)
Pathophysiology The best-supported causal chain is infection → molecular mimicry → anti-ganglioside antibody generation → complement activation at nodes/paranodes/axolemma → conduction failure and axonal degeneration. Pathology in axonal GBS shows antibody/complement deposition on axolemma and macrophage-associated axonal injury. Serum C5 in the eculizumab phase 3 trial was reduced by 99.99% 1 hour post-dose and remained suppressed through week 5, showing effective target engagement. Latov, 2022, Microorganisms https://doi.org/10.3390/microorganisms10112139 (latov2022campylobacterjejuniinfection pages 2-4, leonhard2024guillain–barrésyndrome pages 5-7, kuwabara2024efficacyandsafety pages 1-2)
Diagnostics Diagnosis is primarily clinical and supported by CSF and electrophysiology. For axonal variants, serial NCS/EMG are important because early studies may show reduced CMAPs, reversible conduction failure/block, or equivocal findings; CSF albuminocytologic dissociation is common but may lag. CSF albuminocytologic dissociation in almost 90%; CSF protein elevated in ~70% by end of week 2; electrophysiology more reliable at 3–6 weeks than 1–2 weeks. Shang, 2021, Journal of Neurology https://doi.org/10.1007/s00415-020-09742-2 (shang2021axonalvariantsof pages 1-2, busl2023guidelinesforneuroprognostication pages 1-3, leonhard2024guillain–barrésyndrome pages 1-3)
Prognosis Axonal GBS/AMSAN generally has a more severe course and slower recovery than demyelinating GBS. Across GBS, respiratory failure, bulbar weakness, and severe nadir disability are major poor prognostic features; EGOS and EGRIS are used for outcome and ventilation risk prediction. 10–30% require mechanical ventilation; ~20% of treated GBS patients cannot walk at 6 months; ~5% die; mortality can reach up to 20% among ventilated patients. Busl, 2023, Neurocritical Care https://doi.org/10.1007/s12028-023-01707-3 (busl2023guidelinesforneuroprognostication pages 1-3, leonhard2024guillain–barrésyndrome pages 1-3, umar2024complexneurologicalsequelae pages 4-6)
Treatment Standard evidence-based treatment remains IVIG or plasma exchange; combination therapy and repeat IVIG do not add benefit and can increase adverse events. Supportive ICU care and rehabilitation remain essential, especially in severe axonal cases. Median time to walk without aid: 51 days with IVIG, 49 days with plasma exchange, 40 days with combined treatment in older trial data; repeat IVIG used in 39.7% and combination therapy in 6.1% of a US real-world cohort before newer evidence/guidelines. Kuwabara, 2024, Journal of the Peripheral Nervous System https://doi.org/10.1111/jns.12646 (kuwabara2024efficacyandsafety pages 1-2, stino2024intravenousimmunoglobulinand pages 1-2)
Recent developments 2023-2024 Recent work emphasized 2023 EAN/PNS guideline-based care and mechanism-directed therapy. A 2024 phase 3 trial of eculizumab added to IVIG in severe GBS did not meet its primary endpoint despite strong complement suppression and acceptable safety. Phase 3 eculizumab trial enrolled 57 participants (37 eculizumab, 20 placebo); primary endpoint HR 0.9, 95% CI 0.45–1.97, p=.89. Kuwabara, 2024, Journal of the Peripheral Nervous System https://doi.org/10.1111/jns.12646 (kuwabara2024efficacyandsafety pages 1-2, freiha2026guillainbarrésyndromeprogress pages 7-9)
Real-world implementation Real-world US prescribing data show persistent use of non-recommended repeat IVIG and some IVIG/PLEX combination therapy, suggesting a gap between evidence/guidelines and practice. Diagnostic reclassification from GBS to CIDP also occurs frequently in claims-based care pathways. US cohort n=2325; repeat IVIG 39.7%; combination therapy 6.1%; later reclassified to CIDP 32.0%. Stino, 2024, Muscle & Nerve https://doi.org/10.1002/mus.28265 (stino2024intravenousimmunoglobulinand pages 1-2)

Table: This table condenses the most supported AMSAN findings from the available context into disease knowledge base fields. It highlights what is known specifically for axonal GBS/AMSAN and where evidence comes from broader GBS literature used to inform AMSAN care.

Limitations of this report (evidence availability constraints)

  • AMSAN-specific ontology identifiers (MONDO, Orphanet, OMIM, MeSH term strings) were not available in the retrieved full-text excerpts and therefore are not asserted here.
  • Many epidemiologic and prognosis statistics are reported at the GBS umbrella level; AMSAN-specific incidence, subtype-specific mortality, and antibody-frequency distributions require additional targeted cohort studies not captured in the retrieved evidence subset.

References

  1. (shang2021axonalvariantsof pages 1-2): Pei Shang, Mingqin Zhu, Ying Wang, Xiang-yu Zheng, Xiujuan Wu, Jie Zhu, Jiachun Feng, and Hong-Liang Zhang. Axonal variants of guillain–barré syndrome: an update. Journal of Neurology, pages 1-18, Mar 2021. URL: https://doi.org/10.1007/s00415-020-09742-2, doi:10.1007/s00415-020-09742-2. This article has 93 citations and is from a domain leading peer-reviewed journal.

  2. (leonhard2024guillain–barrésyndrome pages 1-3): Sonja E. Leonhard, Nowshin Papri, Luis Querol, Simon Rinaldi, Nortina Shahrizaila, and Bart C. Jacobs. Guillain–barré syndrome. Nature Reviews Disease Primers, Dec 2024. URL: https://doi.org/10.1038/s41572-024-00580-4, doi:10.1038/s41572-024-00580-4. This article has 48 citations.

  3. (stino2024intravenousimmunoglobulinand pages 1-2): Amro M. Stino, Evan L. Reynolds, Maya Watanabe, and Brian C. Callaghan. Intravenous immunoglobulin and plasma exchange prescribing patterns for guillain‐barre syndrome in the united states—2001 to 2018. Muscle & Nerve, 70:1192-1199, Sep 2024. URL: https://doi.org/10.1002/mus.28265, doi:10.1002/mus.28265. This article has 3 citations and is from a peer-reviewed journal.

  4. (geng2023acutemotorsensoryaxonal pages 3-5): Na Geng, Pengfei Wang, and Yong Zhang. Acute motor-sensory axonal polyneuropathy variant of guillain-barré syndrome with a thalamic lesion and covid-19: a case report and discussion on mechanism. Frontiers in Neurology, Sep 2023. URL: https://doi.org/10.3389/fneur.2023.1227505, doi:10.3389/fneur.2023.1227505. This article has 6 citations and is from a peer-reviewed journal.

  5. (umar2024complexneurologicalsequelae pages 4-6): Anam Umar, Amber E Faquih, Bilal Jawed, and Muhammad Bilal. Complex neurological sequelae: axonal guillain-barré syndrome post covid-19 in a young patient. Cureus, Aug 2024. URL: https://doi.org/10.7759/cureus.67213, doi:10.7759/cureus.67213. This article has 0 citations.

  6. (latov2022campylobacterjejuniinfection pages 1-2): Norman Latov. Campylobacter jejuni infection, anti-ganglioside antibodies, and neuropathy. Microorganisms, 10:2139, Oct 2022. URL: https://doi.org/10.3390/microorganisms10112139, doi:10.3390/microorganisms10112139. This article has 25 citations.

  7. (geng2023acutemotorsensoryaxonal pages 5-6): Na Geng, Pengfei Wang, and Yong Zhang. Acute motor-sensory axonal polyneuropathy variant of guillain-barré syndrome with a thalamic lesion and covid-19: a case report and discussion on mechanism. Frontiers in Neurology, Sep 2023. URL: https://doi.org/10.3389/fneur.2023.1227505, doi:10.3389/fneur.2023.1227505. This article has 6 citations and is from a peer-reviewed journal.

  8. (busl2023guidelinesforneuroprognostication pages 1-3): Katharina M. Busl, Herbert Fried, Susanne Muehlschlegel, Katja E. Wartenberg, Venkatakrishna Rajajee, Sheila A. Alexander, Claire J. Creutzfeldt, Gabriel V. Fontaine, Sara E. Hocker, David Y. Hwang, Keri S. Kim, Dominik Madzar, Dea Mahanes, Shraddha Mainali, Juergen Meixensberger, Oliver W. Sakowitz, Panayiotis N. Varelas, Thomas Westermaier, and Christian Weimar. Guidelines for neuroprognostication in adults with guillain–barré syndrome. Neurocritical Care, 38:564-583, Mar 2023. URL: https://doi.org/10.1007/s12028-023-01707-3, doi:10.1007/s12028-023-01707-3. This article has 52 citations and is from a peer-reviewed journal.

  9. (shang2021axonalvariantsof pages 6-7): Pei Shang, Mingqin Zhu, Ying Wang, Xiang-yu Zheng, Xiujuan Wu, Jie Zhu, Jiachun Feng, and Hong-Liang Zhang. Axonal variants of guillain–barré syndrome: an update. Journal of Neurology, pages 1-18, Mar 2021. URL: https://doi.org/10.1007/s00415-020-09742-2, doi:10.1007/s00415-020-09742-2. This article has 93 citations and is from a domain leading peer-reviewed journal.

  10. (leonhard2024guillain–barrésyndrome pages 5-7): Sonja E. Leonhard, Nowshin Papri, Luis Querol, Simon Rinaldi, Nortina Shahrizaila, and Bart C. Jacobs. Guillain–barré syndrome. Nature Reviews Disease Primers, Dec 2024. URL: https://doi.org/10.1038/s41572-024-00580-4, doi:10.1038/s41572-024-00580-4. This article has 48 citations.

  11. (latov2022campylobacterjejuniinfection pages 2-4): Norman Latov. Campylobacter jejuni infection, anti-ganglioside antibodies, and neuropathy. Microorganisms, 10:2139, Oct 2022. URL: https://doi.org/10.3390/microorganisms10112139, doi:10.3390/microorganisms10112139. This article has 25 citations.

  12. (restrepojimenez2018theimmunotherapyof pages 46-47): Paula Restrepo-Jiménez, Yhojan Rodríguez, Paulina González, Christopher Chang, M. Eric Gershwin, and Juan-Manuel Anaya. The immunotherapy of guillain-barré syndrome. Expert Opinion on Biological Therapy, 18:619-631, May 2018. URL: https://doi.org/10.1080/14712598.2018.1468885, doi:10.1080/14712598.2018.1468885. This article has 26 citations and is from a peer-reviewed journal.

  13. (leonhard2024guillain–barrésyndrome pages 4-5): Sonja E. Leonhard, Nowshin Papri, Luis Querol, Simon Rinaldi, Nortina Shahrizaila, and Bart C. Jacobs. Guillain–barré syndrome. Nature Reviews Disease Primers, Dec 2024. URL: https://doi.org/10.1038/s41572-024-00580-4, doi:10.1038/s41572-024-00580-4. This article has 48 citations.

  14. (kuwabara2024efficacyandsafety pages 1-2): Satoshi Kuwabara, Susumu Kusunoki, Motoi Kuwahara, Yoshihisa Yamano, Yoichiro Nishida, Hirokazu Ishida, Tomoyuki Kasuya, Erik Kupperman, Qun Lin, Glen Frick, and Sonoko Misawa. Efficacy and safety of eculizumab in guillain‐barré syndrome: a phase 3, multicenter, double‐blind, randomized, placebo‐controlled clinical trial. Journal of the Peripheral Nervous System, 29:339-349, Jul 2024. URL: https://doi.org/10.1111/jns.12646, doi:10.1111/jns.12646. This article has 29 citations and is from a peer-reviewed journal.

  15. (freiha2026guillainbarrésyndromeprogress pages 7-9): Joumana Freiha, Young Gi Min, Chinar Osman, and Yusuf Rajabally. Guillain-barré syndrome: progress in diagnosis, biomarkers, neuroimaging and management. ImmunoTargets and Therapy, Volume 15:1-13, Apr 2026. URL: https://doi.org/10.2147/itt.s390161, doi:10.2147/itt.s390161. This article has 0 citations.