GBS
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"Guillain-Barre syndrome"[MeSH Terms] AND treatment
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Guillain-Barré Syndrome (GBS)
Definition
GBS refers to a group of acquired, immune-mediated acute inflammatory polyradiculoneuropathies sharing: acute onset, elevated CSF protein with low cell counts (cytoalbuminologic/albuminocytologic dissociation), and a monophasic course. It is the leading cause of acute paralytic disease in Western countries since the eradication of polio.
- Bradley and Daroff's Neurology in Clinical Practice, p. 2663
- Goldman-Cecil Medicine, p. block 56
Epidemiology
- Annual incidence: 1-2 per 100,000
- Males > females (ratio ~1.4-1.5:1)
- All age groups; incidence rises with age (0.8/100,000 in <18 yr vs. 3.2/100,000 in >60 yr)
- In 60% of patients, a respiratory or gastrointestinal infection precedes GBS by 1-4 weeks
- Mortality: ~3% (down from 33% before the era of positive-pressure ventilation)
Subtypes & Variants
| Subtype | Key Features |
|---|---|
| AIDP (Acute Inflammatory Demyelinating Polyradiculoneuropathy) | Most common in North America/Europe (97%); demyelinating pattern on NCS |
| AMAN (Acute Motor Axonal Neuropathy) | Pure motor; first described in northern China; summer epidemics in children |
| AMSAN (Acute Motor-Sensory Axonal Neuropathy) | Motor + sensory axonal; typically more severe, poorer recovery |
| Miller-Fisher Syndrome (MFS) | Triad: ophthalmoplegia + ataxia + areflexia; anti-GQ1b antibodies in >85%; normal NCS |
| Other rare variants | Pharyngeal-cervical-brachial, facial diplegia with paresthesias, acute pandysautonomia, paraparetic variant |
Precipitating Infections
| Antecedent Event | % Cases |
|---|---|
| Respiratory illness | 58% |
| GI illness (esp. Campylobacter jejuni) | 22% |
| Both respiratory + GI | 10% |
| Surgery | 5% |
| Vaccination | 3% |
Specific organisms: C. jejuni (especially axonal subtypes), CMV, EBV, HIV, Mycoplasma pneumoniae, Hepatitis A/B, Zika virus (significantly elevated risk).
Note: GBS does not appear substantially associated with SARS-CoV-2 infection or vaccination, and GBS incidence actually declined during the COVID-19 pandemic.
Pathophysiology
The underlying mechanism is molecular mimicry: a preceding infection triggers an immune response against peripheral nerve antigens that share epitopes with microbial antigens. Both cellular and humoral arms are involved:
- T-cell mediated: activated T-cells (releasing IL-6, IL-2, IFN-γ, TNF-α) open the blood-nerve barrier, allowing antibodies access to nerve antigens
- Antibody + complement mediated: In AIDP, complement activation products deposit on the outer Schwann cell surface, leading to vesicular myelin changes and macrophage-mediated demyelination. In AMAN/MFS, specific antiganglioside antibodies (anti-GM1, anti-GD1a in AMAN; anti-GQ1b in MFS) attack axonal/nodal membranes
Clinical Features
Classic presentation (AIDP):
- Symmetric ascending weakness beginning in the legs, spreading to arms, face, bulbar muscles, and potentially respiratory muscles
- Areflexia or hyporeflexia (invariable; may be absent early)
- Mild sensory symptoms (paresthesias, distal vibration loss); sensory loss is NOT prominent
- Pain (70% during acute phase) - burning/tingling, back/extremity pain
- Cranial nerve involvement in 45-75% (bilateral facial palsy in ~50%)
- Autonomic dysfunction in ~65%: orthostatic hypotension, urinary retention, ileus, tachyarrhythmias, bradycardia, asystole (vagal)
- Respiratory failure requiring ventilation in 9-30%
- Progression peaks at 2-4 weeks (50% at 2 wk, 75% at 3 wk, >90% at 4 wk)
Diagnostic Criteria
Required:
- Progressive weakness of both legs and arms
- Areflexia or hyporeflexia
Supportive clinical features:
- Progression over days to 4 weeks
- Relative symmetry
- Mild sensory symptoms/signs
- Bifacial palsies, autonomic dysfunction
- Absence of fever at onset
- Recovery beginning 2-4 weeks after progression ceases
Laboratory support:
- CSF: High protein, <10 cells/μL (cytoalbuminologic dissociation); CSF protein may be normal in up to 10% in first 7-10 days
- NCS/EMG: Slowed conduction velocities or conduction block (AIDP); reduced CMAP amplitudes (axonal variants)
- Anti-GQ1b antibodies in Miller-Fisher syndrome (>85%)
CSF WBC >50/μL should prompt consideration of HIV seroconversion or CNS infection rather than GBS.
Differential Diagnosis (Warning Signs)
| Warning Sign | Alternate Diagnosis |
|---|---|
| Sensory predominant | Sensory neuronopathy |
| Bowel/bladder symptoms + sensory level | Myelopathy (transverse myelitis) |
| Persistently asymmetric weakness | Enteroviral encephalomyelitis, vasculitic mononeuritis multiplex |
| Slow progression (>4 wks) | CIDP |
| CSF >50 WBCs/μL | HIV seroconversion, CNS infection |
| Ophthalmoplegia + unreactive pupils + dry mouth | Botulism |
| Brisk reflexes present | CNS lesion (cord compression, brainstem infarct) |
Also consider: myasthenia gravis, critical illness polyneuropathy, acute porphyria, tick paralysis, arsenic/thallium toxicity.
Treatment
Two treatments of proven efficacy - neither is superior to the other, and combining them is not better than either alone:
- IV Immunoglobulin (IVIG) - 0.4 g/kg/day for 5 days (total 2 g/kg)
- Plasmapheresis (plasma exchange) - typically 4-6 exchanges over 1-2 weeks
Indications for treatment: Non-ambulatory patients, rapidly progressive weakness, respiratory compromise.
Corticosteroids - NOT beneficial and may worsen outcome; not recommended.
Supportive care is critical:
- Respiratory monitoring (FVC, NIF/MIP); intubate if FVC <15-20 mL/kg or NIF >-25 cmH2O ("20-30-40 rule")
- DVT prophylaxis
- Pain management
- Cardiac monitoring for autonomic instability
- Nutrition and physical rehabilitation
Prognosis
- 85% recover functional ambulation
- 20% have persistent disability
- Mortality ~3% at 6 months
- Poorer prognosis: older age, preceding diarrheal illness (C. jejuni), axonal variants, severe weakness at nadir, reduced CMAP amplitudes in upper limbs
Recent evidence (2025-2026): A systematic review (PMID 41038424) confirmed that structured exercise programs improve fatigue and functional capacity in GBS and CIDP patients during the rehabilitation phase.
Sources: Bradley and Daroff's Neurology in Clinical Practice; Goldman-Cecil Medicine, 2-Vol Set; Miller's Anesthesia, 10e; Robbins & Kumar Basic Pathology
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