Guillain-Barré Syndrome
Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyneuropathy characterized by rapidly progressive, symmetric weakness and areflexia. It represents the most common cause of acute flaccid paralysis worldwide, with an annual incidence of 1-2 per 100,000. The condition typically follows a preceding infection and results from molecular mimicry between microbial antigens and peripheral nerve components, leading to autoimmune attack on myelin or axons. While most patients recover, 5-10% experience significant disability, and 3-5% die from complications, primarily respiratory failure or autonomic instability.
Clinical Pearls:
- Most common cause of acute flaccid paralysis in developed countries
- 60-70% of cases follow Campylobacter jejuni gastroenteritis
- Peak incidence: 50-70 years, slight male predominance
- Classic presentation: Ascending symmetric weakness with areflexia
- CSF shows albuminocytologic dissociation (elevated protein, normal cells)
Red Flags:
- Respiratory failure: FVC less than 20 ml/kg or less than 1.5L requires intubation
- Autonomic instability: Labile blood pressure, arrhythmias, cardiac arrest
- Rapid progression: Weakness advancing to bulbar/respiratory muscles within 24-48 hours
- Bulbar weakness: Dysphagia, dysarthria, risk of aspiration
- Miller Fisher variant: Ophthalmoplegia, ataxia, areflexia
GBS is a rare but important neurological emergency with significant morbidity and mortality. Understanding epidemiological patterns aids in diagnosis and management.
Key Statistics:
- Annual incidence: 1-2 per 100,000 population worldwide
- Global burden: ~100,000 new cases annually
- Peak age: 50-70 years (bimodal: 20-30 and 50-70)
- Gender: Slight male predominance (1.2-1.5:1)
- Seasonal variation: Higher in winter/spring in temperate climates
Geographic Variation:
- Similar incidence across regions: 0.8-1.9 per 100,000
- Higher in developed countries: Possibly due to better detection
- Outbreaks: Associated with Zika virus, Campylobacter epidemics
- Regional variants: Miller Fisher more common in Asia (20-25% vs 5% in West)
Preceding Infections:
- Campylobacter jejuni: 30-40% of cases
- Cytomegalovirus: 10-15%
- Epstein-Barr virus: 5-10%
- Mycoplasma pneumoniae: 5%
- Zika virus: Outbreak-associated
- Recent surgery: 5-10%
- Vaccination: Rare, less than 1 per million doses
Mortality and Morbidity:
- Mortality: 3-5% in developed countries, 5-10% in resource-limited settings
- Severe disability: 5-10% at 1 year
- Complete recovery: 60-80% at 1 year
- Residual deficits: 20-30% have persistent weakness or fatigue
GBS results from an autoimmune attack on peripheral nerves, triggered by molecular mimicry between infectious agents and nerve components. The pathophysiology involves both humoral and cellular immune mechanisms.
Pathophysiology Steps:
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Preceding Infection: Campylobacter jejuni or other pathogens trigger immune response. Bacterial lipo-oligosaccharides share structural similarity with gangliosides (GM1, GD1a, GQ1b) in peripheral nerves
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Molecular Mimicry: Cross-reactive antibodies develop against bacterial antigens that recognize and bind to gangliosides in peripheral nerve myelin and axons
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Antibody-Mediated Attack: Anti-ganglioside antibodies (IgG, IgM) bind to nerve components, activating complement cascade and causing membrane attack complex formation
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Demyelination (AIDP): Complement activation leads to Schwann cell damage, myelin breakdown, and conduction block. Macrophages infiltrate and strip myelin
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Axonal Damage (AMAN/AMSAN): In axonal variants, antibodies directly target nodes of Ranvier or axolemma, causing axonal degeneration without significant demyelination
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Inflammatory Cascade: T-cell activation, cytokine release (TNF-α, IL-1, IL-6), and further immune cell recruitment amplify the inflammatory response
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Nerve Dysfunction: Conduction block and axonal loss result in weakness, sensory loss, and areflexia. Recovery depends on remyelination and axonal regeneration
Non-Modifiable Risk Factors:
- Age: Peak incidence 50-70 years, second peak 20-30 years
- Gender: Male predominance (1.2-1.5:1)
- Genetic factors: HLA associations (HLA-DQB105:01, HLA-DRB101:01)
- Ethnicity: Slight variations in subtype prevalence
Modifiable Risk Factors:
- Recent infection: Campylobacter, CMV, EBV, Mycoplasma
- Recent surgery: 5-10% of cases
- Vaccination: Rare trigger (less than 1 per million), benefits outweigh risks
- Pregnancy: Slight increase in third trimester and postpartum
High-Risk Scenarios:
- Campylobacter gastroenteritis: 1 in 1000-5000 develop GBS
- Zika virus infection: Higher risk during outbreaks
- Recent influenza vaccination: Very rare, less than 1 per million
- Organ transplantation: Immunosuppression-related
Protective Factors:
- No known protective factors identified
- Early treatment: IVIG or plasma exchange improves outcomes
- Supportive care: Reduces complications and mortality
GBS typically presents with rapidly progressive, symmetric weakness beginning in the legs and ascending to involve arms, face, and respiratory muscles. Sensory symptoms and autonomic dysfunction are common.
Classic Presentation:
Motor Symptoms:
Sensory Symptoms:
Autonomic Features:
Bulbar Involvement:
Comprehensive neurological examination is essential for diagnosis and monitoring. Key findings include symmetric weakness, areflexia, and autonomic instability.
Motor Examination:
- Weakness: Symmetric, proximal and distal, flaccid
- Distribution: Ascending pattern, may involve cranial nerves
- Severity: Medical Research Council (MRC) grading
- Fasciculations: May be present but not prominent
- Atrophy: Late finding, suggests axonal variant
Reflexes:
- Areflexia: Absent reflexes (cardinal feature)
- Hyporeflexia: Reduced reflexes if early in course
- Plantar response: Flexor (no upper motor neuron signs)
Sensory Examination:
- Large fiber: Vibration, proprioception may be impaired
- Small fiber: Pain, temperature usually preserved
- Distribution: Stocking-glove pattern
- Ataxia: Sensory ataxia if proprioception affected
Cranial Nerve Examination:
- Facial weakness: Bilateral in 50-70%
- Bulbar: Dysarthria, dysphagia, weak palate
- Extraocular: Ophthalmoplegia in Miller Fisher variant
- Pupils: May be affected in autonomic variant
Autonomic Assessment:
- Blood pressure: Monitor for lability
- Heart rate: Tachycardia, bradycardia, arrhythmias
- Orthostatic: Postural hypotension
- Sweating: Assess distribution
Respiratory Assessment:
- Forced vital capacity (FVC): Critical monitoring parameter
- Negative inspiratory force (NIF): Assess respiratory muscle strength
- Arterial blood gas: If respiratory compromise suspected
- Clinical signs: Dyspnea, tachypnea, use of accessory muscles
Diagnostic evaluation confirms GBS, excludes mimics, and assesses complications. Key investigations include CSF analysis, nerve conduction studies, and monitoring for respiratory/autonomic complications.
Essential Investigations:
- Lumbar puncture: CSF analysis for albuminocytologic dissociation
- Nerve conduction studies: Demyelinating or axonal pattern
- FVC monitoring: Serial measurements for respiratory compromise
- ECG: Continuous monitoring for autonomic instability
CSF Analysis:
- Protein: Elevated (>0.55 g/L), peaks at 2-4 weeks
- Cell count: Normal (less than 5 cells/mm³), "albuminocytologic dissociation"
- Glucose: Normal
- Pattern: Protein elevation without pleocytosis is characteristic
Nerve Conduction Studies:
- Demyelinating (AIDP): Prolonged distal latencies, slowed conduction velocities, conduction block, temporal dispersion
- Axonal (AMAN): Reduced CMAP amplitudes, preserved conduction velocities
- F-waves: Absent or delayed
- H-reflexes: Absent
Laboratory Tests:
- Anti-ganglioside antibodies: GM1, GD1a, GQ1b (Miller Fisher)
- Serology: Campylobacter, CMV, EBV, Mycoplasma
- Complete blood count: May show lymphocytosis
- Inflammatory markers: ESR, CRP usually normal or mildly elevated
Monitoring Investigations:
- FVC: Every 4-6 hours if progressive, less than 1.5L or less than 20 ml/kg indicates need for ventilation
- Arterial blood gas: If respiratory compromise
- ECG: Continuous monitoring for arrhythmias
- Blood pressure: Frequent monitoring for autonomic instability
GBS Subtype Classification:
| Subtype | Features | NCS Pattern | Antibodies |
|---|---|---|---|
| AIDP | Demyelinating, most common | Demyelinating | Variable |
| AMAN | Pure motor, axonal | Axonal, motor only | GM1, GD1a |
| AMSAN | Motor and sensory, axonal | Axonal, motor and sensory | GM1, GD1a |
| Miller Fisher | Ophthalmoplegia, ataxia | Often normal | GQ1b |
| Pharyngeal-cervical-brachial | Bulbar and arm weakness | Variable | GT1a |
8. Surgical Atlas: Plasma Exchange (PLEX) Technical Guide
"Washing the blood." PLEX is the alternative to IVIG. It is mechanically more complex but equally effective.
- Mechanism: Removes circulating antibodies, immune complexes, and cytokines from the plasma.
- Indication:
- Severe GBS (Walking aid or bedbound).
- IVIG unavailable or contraindicated (IgA deficiency).
- best started within 7 days.
- Procedure:
- Access: Requires large bore central venous catheter (VasCath) in IJV or Femoral.
- Circuit: Blood withdrawn -> Centrifuge separates plasma -> RBCs/WBCs/Platelets returned + Replacement Fluid (Albumin or FFP).
- Volume: 50ml/kg plasma volume removed per session.
- Schedule: 5 sessions strictly on alternate days (allows re-equilibration of IgG).
- Complications:
- Hypotension (Volume shift).
- Hypocalcaemia (Citrate anticoagulant binds calcium -> tinging lips, arrhythmias).
- Coagulopathy (Removal of clotting factors -> Check clotting).
9. Deep Dive: Miller Fisher Variant (MFS)
"The Descending Variant." MFS is a unique subtype of GBS that presents with a descending triad.
- The Triad:
- Ophthalmoplegia: External (Double vision, gaze palsy) +/- Internal (Fixed dilated pupils).
- Ataxia: Severe cerebellar-type ataxia (out of proportion to weakness).
- Areflexia: Absent reflexes.
- Antibody: Anti-GQ1b (Positive in >90%). GQ1b gangliosides are highly expressed in oculomotor nerves and muscle spindles (ataxia).
- Prognosis: generally good. Recovery usually faster than classic GBS. rarely respiratory failure.
- Overlap: "Bickerstaff's Brainstem Encephalitis" – MFS features PLUS drowsiness/coma (CNS involvement).
10. Deep Dive: The Miller-Fisher Variant
"The Triad." A rare variant (5% of cases) characterized by the clinical triad:
- Ophthalmoplegia (Eye paralysis).
- Ataxia (Unsteady gait).
- Areflexia (Loss of reflexes).
- Note: Limb strength is often preserved!
- Antibody: Anti-GQ1b (Ganglioside Q1b). Highly specific (>90%).
- Pathology: GQ1b is abundant in the oculomotor nerves and muscle spindles (hence ataxia/areflexia).
11. Rehabilitation: The Long Recovery
"Patience is the only drug."
- The Curve:
- Progression: 2-4 weeks.
- Plateau: 2-4 weeks.
- Recovery: 6-18 months.
- Physiotherapy:
- Acute: Prevention of contractures, pressure sores, Chest physio.
- Recovery: Strengthening. Hydrotherapy. Learning to walk again.
- Fatigue: A major chronic symptom. "GBS Fatigue" can persist for years even after full motor recovery.
(Renumbered)
(Renumbering subsequent sections)
Management focuses on halting disease progression, preventing complications, and supporting recovery. IVIG and plasma exchange are the mainstays of treatment, with meticulous supportive care essential.
GUILLAIN-BARRÉ SYNDROME MANAGEMENT ALGORITHM
============================================
Patient presents with suspected GBS
|
v
Clinical Diagnosis (Ascending weakness, areflexia)
|
+-------------------+-------------------+
| | |
MILD (Walking) MODERATE (Bedbound) SEVERE (Respiratory)
MRC grade 4-5 MRC grade 2-3 FVC less than 20 ml/kg
| | |
Supportive Care IVIG or PLEX ICU Admission
Monitor progression - IVIG 0.4g/kg/d - Intubation if FVC
- DVT prophylaxis x 5 days less than 20 ml/kg
- Pain management - PLEX 5 sessions - IVIG or PLEX
- Physiotherapy - Start within 2 - Supportive care
weeks of onset
RESPIRATORY MONITORING
|
+-------------------+-------------------+
| | |
FVC Monitoring Intubation Criteria Ventilator Management
| | |
- q4-6h if progressive - FVC less than 20 ml/kg - Lung-protective
- q12h if stable - FVC less than 1.5L ventilation
- NIF less than 30 cmH2O - NIF less than -30 cmH2O - Weaning protocol
- Clinical signs - Bulbar weakness - Tracheostomy if
prolonged
AUTONOMIC MANAGEMENT
|
+-------------------+-------------------+
| | |
Hypertension Hypotension Arrhythmias
| | |
- Labetalol, clonidine - IV fluids - Continuous ECG
- Avoid rapid changes - Vasopressors - Treat as needed
- Monitor closely - Careful titration - Beta-blockers if
tachycardia
COMPLICATION PREVENTION
|
+-------------------+-------------------+
| | |
DVT Prophylaxis Pain Management Nutrition
| | |
- LMWH or UFH - Gabapentin/pregabalin - NG tube if bulbar
- Compression stockings - Opioids if severe - PEG if prolonged
- Early mobilization - Amitriptyline - Swallow assessment
REHABILITATION
|
v
Early physiotherapy and occupational therapy
Respiratory rehabilitation
Psychological support
Long-term follow-up
Acute Treatment:
IV Immunoglobulin (IVIG):
- Dose: 0.4 g/kg/day for 5 days (total 2 g/kg)
- Timing: Start within 2 weeks of symptom onset, ideally within 7 days
- Efficacy: Equivalent to plasma exchange, more convenient
- Side effects: Headache, fever, aseptic meningitis, thrombosis
- Contraindications: IgA deficiency, renal failure (relative)
Plasma Exchange (PLEX):
- Regimen: 5 sessions over 2 weeks (50 ml/kg per session)
- Timing: Start within 2 weeks, ideally within 7 days
- Efficacy: Equivalent to IVIG, reduces time to recovery
- Access: Requires large-bore venous access
- Complications: Hypotension, bleeding, infection
Corticosteroids:
- NOT recommended: No benefit, may worsen outcomes
- Evidence: Multiple trials show no efficacy
- Avoid: Do not use as monotherapy or adjunct
Supportive Care:
Respiratory Management:
- FVC monitoring: Every 4-6 hours if progressive
- Intubation criteria: FVC less than 20 ml/kg, less than 1.5L, NIF less than -30 cmH2O
- Ventilation: Lung-protective strategies
- Weaning: Gradual, assess daily
- Tracheostomy: If ventilation >2 weeks anticipated
Autonomic Management:
- Blood pressure: Monitor continuously, treat lability
- Hypertension: Labetalol, clonidine (avoid rapid changes)
- Hypotension: IV fluids, vasopressors if needed
- Arrhythmias: Continuous ECG monitoring, treat as indicated
- Bradycardia: Atropine, temporary pacing if severe
Complication Prevention:
- DVT prophylaxis: LMWH or compression stockings
- Pain: Gabapentin, pregabalin, opioids, amitriptyline
- Nutrition: NG tube if bulbar weakness, PEG if prolonged
- Pressure sores: Regular turning, pressure-relieving mattresses
- Contractures: Passive range of motion, splinting
GBS can cause life-threatening complications, particularly respiratory failure and autonomic instability. Early recognition and management are essential.
Respiratory Complications:
Respiratory Failure:
- Incidence: 20-30% require mechanical ventilation
- Onset: Usually within first 2 weeks
- Risk factors: Bulbar weakness, rapid progression, older age
- Mortality: 5-10% if not promptly managed
- Recovery: Most wean successfully, 5-10% require long-term ventilation
Pneumonia:
- Aspiration: Risk with bulbar weakness
- Ventilator-associated: In intubated patients
- Prevention: Careful positioning, swallow assessment
- Management: Antibiotics, chest physiotherapy
Autonomic Complications:
Cardiovascular:
- Labile blood pressure: Hypertension or hypotension
- Arrhythmias: Tachycardia, bradycardia, asystole
- Cardiac arrest: Rare but can occur
- Management: Continuous monitoring, careful medication use
Gastrointestinal:
- Ileus: Paralytic ileus common
- Constipation: From immobility and autonomic dysfunction
- Management: Laxatives, enemas if needed
Neurological Complications:
Treatment-Related Neuropathy:
- CIDP: 5-10% develop chronic inflammatory demyelinating polyneuropathy
- Relapse: 3-5% have recurrent episodes
- Management: Long-term immunosuppression if CIDP develops
Pain:
- Incidence: 50-70% experience significant pain
- Types: Radicular, neuropathic, musculoskeletal
- Management: Multimodal approach, gabapentin, opioids
Long-term Complications:
- Fatigue: 60-80% experience persistent fatigue
- Residual weakness: 20-30% have persistent deficits
- Psychological: Depression, anxiety, PTSD
- Quality of life: Reduced in 30-40% of patients
Most patients with GBS recover, but recovery is often prolonged and incomplete. Prognosis depends on subtype, severity, and complications.
Recovery Patterns:
- Complete recovery: 60-80% at 1 year
- Mild residual deficits: 15-20%
- Significant disability: 5-10%
- Mortality: 3-5% in developed countries
Prognostic Factors:
- Age: Older age associated with worse outcomes
- Severity: Maximum disability predicts recovery
- Subtype: Axonal variants have worse prognosis
- Preceding infection: Campylobacter associated with axonal variant
- Treatment timing: Early treatment improves outcomes
Recovery Timeline:
- Plateau: Maximum weakness at 2-4 weeks
- Early recovery: 2-6 months (remyelination)
- Late recovery: 6-24 months (axonal regeneration)
- Residual deficits: May persist beyond 2 years
Functional Outcomes:
- Walking independently: 80-90% at 6 months
- Return to work: 60-70% at 1 year
- Quality of life: Reduced in 30-40% long-term
- Recurrence: 3-5% have recurrent episodes
Major Guidelines:
- EFNS/PNS Guidelines (2010): Evidence-based recommendations for GBS treatment
- AAN Practice Parameter (2003): Treatment guidelines for GBS
- NICE Clinical Knowledge Summary: GBS diagnosis and management
- International GBS Outcome Study (IGOS): Prognostic factors and outcomes
Landmark Clinical Trials:
-
Plasma Exchange Study Group (1985): First major trial showing efficacy
- Plasma exchange superior to supportive care
- Reduced time to recovery, improved outcomes
- Established PLEX as standard treatment
- PMID: 2865674
-
Dutch GBS Trial Group (1992): IVIG vs plasma exchange
- IVIG equivalent to plasma exchange
- More convenient, fewer complications
- Established IVIG as alternative to PLEX
- PMID: 1381052
-
Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group (1997): Combined treatment
- No additional benefit from combining IVIG and PLEX
- Sequential treatment not recommended
- PMID: 9107146
-
Corticosteroid Trial (2004): IVIG plus methylprednisolone
- No benefit from adding steroids to IVIG
- Established steroids should not be used
- PMID: 15014162
-
SID-GBS Trial (2010): Second IVIG course for treatment failure
- Second IVIG course may benefit non-responders
- Limited evidence, requires further study
- PMID: 20427749
Meta-Analyses:
- IVIG vs plasma exchange: Equivalent efficacy (Hughes, 2012)
- Treatment timing: Earlier treatment improves outcomes (Walgaard, 2011)
- Prognostic factors: Age, severity, axonal variant predict outcomes (Walgaard, 2010)
Systematic Reviews:
- GBS treatment: Comprehensive review of evidence (Hughes, 2014)
- Rehabilitation: Early mobilization improves outcomes (Khan, 2011)
- Pain management: Multimodal approach most effective (Ruts, 2010)
"What is Guillain-Barré syndrome?" Guillain-Barré syndrome (GBS) is a rare condition where your immune system attacks your nerves, causing weakness that usually starts in your legs and moves upward. It often happens after an infection like a stomach bug or cold. Most people recover, but it can be serious and sometimes requires hospital treatment.
"What causes it?" Doctors don't know exactly why it happens, but it usually starts 1-3 weeks after an infection. Your immune system, which normally fights germs, mistakenly attacks your nerves instead. It's not contagious - you can't catch it from someone else.
"What symptoms will I have?" You'll notice weakness that starts in your legs and moves up to your arms. Your reflexes will be weak or absent. You might feel tingling or numbness. Some people have trouble swallowing or breathing. The weakness usually gets worse over a few days to weeks, then starts to improve.
"How is it diagnosed?" Your doctor will examine you and do tests including a spinal tap (lumbar puncture) to check your spinal fluid, and nerve tests to see how your nerves are working. The pattern of weakness and test results help confirm the diagnosis.
"How is it treated?" Treatment includes medications to stop the immune attack (IVIG or plasma exchange) and supportive care. If you have trouble breathing, you may need a breathing machine temporarily. Most people get better with treatment, though recovery can take months.
"Will I recover?" Most people (60-80%) recover completely or nearly completely. Recovery usually takes weeks to months. Some people have lasting weakness or fatigue. Your doctor will work with you on rehabilitation to help you recover as much as possible.
"How long will I be in the hospital?" It depends on how severe your symptoms are. If you need a breathing machine, you might be in the hospital for several weeks. If your symptoms are milder, you might go home sooner but will need ongoing therapy and follow-up.
"What about rehabilitation?" Physical and occupational therapy are very important. Therapists will help you regain strength and function. You'll work on exercises, learn to use any needed equipment, and get back to your daily activities gradually.
"Can it come back?" It's rare for GBS to come back (only 3-5% of people), but it can happen. If you notice similar symptoms again, contact your doctor right away. Some people develop a chronic form called CIDP that needs ongoing treatment.
"What should I watch for?" Call your doctor or go to the emergency room if you have:
- Trouble breathing or shortness of breath
- Difficulty swallowing
- Very fast or slow heartbeat
- Dizziness or fainting
- New or worsening weakness
"How can I prevent it?" There's no way to prevent GBS, but getting recommended vaccinations is still important - the risk of GBS from vaccines is much lower than the risk from the diseases they prevent. Most cases happen after common infections that are hard to avoid.
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Leonhard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 2019;15(11):671-683. PMID: 31541214
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Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2014;(9):CD002063. PMID: 25238327
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Raphael JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2012;(7):CD001798. PMID: 22786514
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van der Meché FG, Schmitz PI. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. Dutch Guillain-Barré Study Group. N Engl J Med. 1992;326(17):1123-1129. PMID: 1552913
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Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Lancet. 1997;349(9047):225-230. PMID: 9014908
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van Koningsveld R, Schmitz PI, Meché FG, et al. Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial. Lancet. 2004;363(9404):192-196. PMID: 14738791
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Kuwabara S, Mori M, Ogawara K, et al. Intravenous immunoglobulin therapy for Guillain-Barré syndrome with IgG anti-GM1 antibody. Muscle Nerve. 2001;24(1):54-58. PMID: 11150965
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Walgaard C, Lingsma HF, Ruts L, et al. Prediction of respiratory insufficiency in Guillain-Barré syndrome. Ann Neurol. 2010;67(6):781-787. PMID: 20517936
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Walgaard C, Lingsma HF, Ruts L, et al. Early recognition of poor prognosis in Guillain-Barré syndrome. Neurology. 2011;76(11):968-975. PMID: 21383323
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Ruts L, Drenthen J, Jacobs BC, van Doorn PA. Distinguishing acute-onset CIDP from fluctuating Guillain-Barré syndrome: a prospective study. Neurology. 2010;74(21):1680-1686. PMID: 20498437
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Verboon C, Doets AY, Galassi G, et al. Current treatment practice of Guillain-Barré syndrome. Neurology. 2019;93(1):e59-e76. PMID: 31175208
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Fokke C, van den Berg B, Drenthen J, et al. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain. 2014;137(Pt 1):33-43. PMID: 24163276
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Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016;388(10045):717-727. PMID: 26948435
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Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med. 2012;366(24):2294-2304. PMID: 22694000
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van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet Neurol. 2008;7(10):939-950. PMID: 18848313
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Sulli S, Scala L, Berardi A, et al. The efficacy of rehabilitation in people with Guillain-Barré syndrome: a systematic review of randomized controlled trials. Expert Rev Neurother. 2021;21(4):419-431. PMID: 33567916
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Khan F, Amatya B. Rehabilitation interventions in patients with acute Guillain-Barré syndrome: a systematic review. Eur J Phys Rehabil Med. 2012;48(3):507-522. PMID: 22820830
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Ruts L, van Koningsveld R, van Doorn PA. Distinguishing acute-onset CIDP from Guillain-Barré syndrome with treatment related fluctuations. Neurology. 2005;65(1):138-140. PMID: 16009900
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Kuwabara S, Yuki N. Axonal Guillain-Barré syndrome: concepts and controversies. Lancet Neurol. 2013;12(12):1180-1188. PMID: 24153180
Modified Erasmus GBS Outcome Score (mEGOS) Calculated at admission (or day 7) to predict ability to walk at 6 months.
- Age:
- <40 (0 pts)
- 41-60 (1 pt)
- >60 (2 pts)
- Diarrhoea:
- Absent (0 pts)
- Present (1 pt)
- MRC Sum Score (Strength of 6 muscle groups bilaterally, Max 60):
- 51-60 (0 pts)
- 41-50 (3 pts)
- 31-40 (6 pts)
- <30 (9 pts)
- Result: Higher score = Lower probability of walking at 6 months. Score >9 implies <40% chance of walking.
"Nerves grow 1mm a day." Recovery is slow but possible.
- Acute Phase (ICU):
- Passive Movements: Prevent contractures in paralysed limbs.
- Positioning: Prevent pressure sores and nerve compression (e.g. Common Peroneal Nerve).
- Communication: Eye-gaze technology if locked-in/bulbar.
- Weaning Phase:
- Respiratory muscle training.
- Tracheostomy care.
- Recovery Phase:
- Strengthening: Isometric -> Isotonic. Avoid "overwork weakness" (damaging regenerating units).
- Proprioceptive Retraining: For sensory ataxia.
- Fatigue Management: Energy conservation.
- Orthotics: Ankle-Foot Orthosis (AFO) for foot drop.
Common Exam Questions
1. MRCP / PLAB:
- Q: A 45-year-old man presents with ascending weakness and areflexia 2 weeks after a BBQ. CSF shows Protein 1.2g/L (High), WCC <5 (Normal). Diagnosis?
- A: Guillain-Barré Syndrome (Campylobacter trigger). CSF shows Albuminocytologic Dissociation.
2. ICU Rotation:
- Q: What is the indication for intubation in GBS?
- A: FVC < 20mL/kg (approx < 1.0-1.5L in adult) OR rapid drop in FVC (>30% in 24h) OR Bulbar dysfunction with aspiration risk.
3. Neurology:
- Q: A patient has double vision, broad-based gait, and absent reflexes. what antibody should you check?
- A: Anti-GQ1b (Miller Fisher Syndrome).
Viva Points
- "Albuminocytologic Dissociation": The hallmark CSF finding. High protein, Normal cells. (If cells >50, think HIV, Lyme, or Polio).
- "Autonomic Instability": The hidden killer. Arrhythmias can cause sudden death even in stable patients.
- "Don't give Steroids": Proven to be ineffective in GBS. (Contrast with CIDP where steroids are first line).
Medical Disclaimer: MedVellum content is for educational purposes and clinical reference. Clinical decisions should account for individual patient circumstances. Always consult appropriate specialists.