Guillain-Barré Syndrome

Quick Answer

One-liner: Guillain-Barré Syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy causing rapidly progressive ascending weakness, areflexia, and autonomic dysfunction; emergency department priorities include monitoring for respiratory failure (using the 20/30/40 rule), initiating IVIG 2 g/kg over 5 days or plasmapheresis, and admitting to ICU for continuous monitoring.

GBS is the most common cause of acute flaccid paralysis worldwide, affecting 1-2 people per 100,000 annually. Up to 30% of patients develop respiratory failure requiring mechanical ventilation, making early recognition and proactive airway management critical. The classic presentation involves ascending paralysis starting from the lower limbs, diminished or absent reflexes (areflexia), and often antecedent respiratory or gastrointestinal infection. Emergency department management focuses on respiratory monitoring using the 20/30/40 rule (vital capacity below 20 mL/kg, negative inspiratory force < -30 cmH2O, maximal expiratory force < 40 cmH2O), initiating immunotherapy (IVIG 2 g/kg over 5 days OR plasmapheresis), and ICU admission for autonomic instability monitoring. Miller Fisher variant presents with ophthalmoplegia, ataxia, and areflexia, with greater than 90% having anti-GQ1b antibodies. Mortality ranges from 3-7%, primarily from respiratory failure or cardiac complications.

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ACEM Exam Focus

Primary Exam Relevance

Anatomy:

• Peripheral nerve structure: myelin sheath (Schwann cells), nodes of Ranvier, ventral/dorsal roots
• Cranial nerves III, IV, VI (extraocular movements) - Miller Fisher variant targets
• Phrenic nerve and diaphragm innervation (C3-C5) - respiratory failure relevance
• Autonomic nervous system: sympathetic vs parasympathetic pathways

Physiology:

• Neuromuscular transmission: action potential propagation, saltatory conduction
• Muscle spindle function and stretch reflex arc
• Respiratory mechanics: vital capacity, inspiratory/expiratory forces
• Autonomic regulation: baroreceptor reflex, heart rate variability, gastrointestinal motility

Pharmacology:

• IVIG: mechanism of action, dosing (2 g/kg over 5 days), adverse effects (renal failure, thromboembolism, anaphylaxis)
• Plasmapheresis: principles, complications (hypotension, bleeding, catheter infection)
• Neuropathic pain agents: gabapentin, pregabalin, carbamazepine mechanisms
• Steroids: INEFFECTIVE in GBS - important exam point

Fellowship Exam Relevance

Written:

• Brighton diagnostic criteria and interpretation
• Differential diagnosis of acute flaccid paralysis (myasthenia gravis, botulism, tick paralysis, transverse myelitis)
• EGRIS score calculation and interpretation for respiratory failure risk
• CSF interpretation: albuminocytologic dissociation
• Indications for IVIG vs plasmapheresis (equal efficacy, choose based on availability/comorbidities)
• Miller Fisher variant: anti-GQ1b antibodies, ophthalmoplegia, ataxia
• Anti-ganglioside antibodies: GM1, GD1a, GT1a, GQ1b and clinical correlations

OSCE:

• Resuscitation station: GBS patient with declining respiratory parameters, intubation decision
• Examination station: Neurological examination of acute ascending weakness, assessing reflexes, bulbar function
• Communication station: Breaking diagnosis of GBS to patient/family, explaining prognosis, need for ICU admission

Key domains tested:

• Medical Expert: Diagnosis, risk stratification, treatment selection
• Collaborator: Neurology consultation, ICU coordination, multidisciplinary team
• Communicator: Explaining progressive paralysis, prognosis uncertainty, family expectations
• Leader: Triage decisions for ICU vs ward, airway management timing

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Key Points

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Epidemiology

Australian/NZ Specific

Australian incidence: 1.5-2.0 per 100,000 person-years, with approximately 350-500 new cases annually [2]. The incidence is similar across states but presentation may be delayed in remote areas.

Indigenous population: Higher rates of preceding Campylobacter jejuni and other enteric infections in remote communities may increase GBS risk, though specific epidemiological data limited [2]. Indigenous patients often present later with more severe functional impairment due to geographic barriers.

Age distribution: Bimodal distribution with peaks in young adults (20-30 years) and older adults (60-70 years) [1].

Seasonal patterns: Slight winter predominance correlating with increased respiratory and gastrointestinal infections [1].

Pathophysiology

Mechanism: GBS is an acute immune-mediated polyradiculoneuropathy triggered by molecular mimicry. Antecedent infection (most commonly Campylobacter jejuni) produces antibodies that cross-react with peripheral nerve components, causing immune-mediated damage.

Molecular mimicry cascade:

Campylobacter jejuni infection → Bacterial lipooligosaccharide (LOS) mimics human gangliosides →
Production of anti-ganglioside antibodies → Antibody binding to peripheral nerve nodes of Ranvier →
Complement activation (C5b-9 membrane attack complex) → Membrane disruption and conduction block →
Muscle weakness and sensory symptoms

Ganglioside targets:

• GM1/GD1a: Associated with acute motor axonal neuropathy (AMAN), Campylobacter jejuni infection, severe motor weakness [6] PMID: 21696237
• GQ1b: Pathognomonic for Miller Fisher syndrome; greater than 90% of MFS patients positive; targets oculomotor nerves and muscle spindles (ophthalmoplegia, ataxia) [8] PMID: 1530398
• GT1a: Associated with bulbar weakness (dysphagia, dysphonia) [9] PMID: 24706593

Pathological variants:

• AIDP (Acute Inflammatory Demyelinating Polyradiculoneuropathy): 70-80% in Western countries; demyelination with lymphocytic infiltration; conduction block on nerve conduction studies
• AMAN (Acute Motor Axonal Neuropathy): 10-20% in Western countries, higher in Asia/Central America; direct axonal damage; associated with anti-GM1/GD1a antibodies; Campylobacter jejuni trigger; more severe motor deficit, slower recovery [6] PMID: 21696237
• AMSAN (Acute Motor Sensory Axonal Neuropathy): Rare; both motor and sensory axonal damage; most severe variant with poorest prognosis
• Miller Fisher Syndrome: 5-15% of cases; anti-GQ1b antibodies; ophthalmoplegia, ataxia, areflexia; better prognosis than classic GBS [7] PMID: 11431611
• Bickerstaff brainstem encephalitis: Anti-GQ1b positive; ophthalmoplegia, ataxia, impaired consciousness; part of "anti-GQ1b antibody syndrome" spectrum with MFS [9] PMID: 24706593

Clinical Progression

Trigger (1-3 weeks before onset)
        ↓
Progressive ascending weakness (peaks 2-4 weeks)
        ↓
Plateau phase (days to weeks)
        ↓
Recovery phase (weeks to months, may take 12-18+ months)

Key timeframes:

• Onset to peak: Usually 2-4 weeks (range 1 day to 6 weeks)
• Plateau phase: Variable duration, from days to weeks
• Recovery: Most significant recovery in first 6 months; may continue up to 18-24 months
• 20% of patients: Unable to walk independently at 6 months [3] PMID: 15505030

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Clinical Approach

Recognition

Key triggers for considering GBS in the ED:

• Progressive symmetrical weakness (usually ascending from lower limbs)
• Diminished or absent deep tendon reflexes (areflexia/hyporeflexia)
• Recent illness (2-3 weeks prior): respiratory infection, gastroenteritis (especially Campylobacter jejuni), vaccination, surgery
• Bulbar symptoms: dysphagia, dysphonia, facial weakness
• Autonomic symptoms: labile blood pressure, tachycardia/bradycardia, urinary retention, ileus
• Pain: neuropathic pain (often severe, affecting back, buttocks, limbs) in up to 89% of patients

Red flags for immediate concern:

• Rapidly progressive weakness (inability to walk within 24 hours)
• Respiratory distress, tachypnea greater than 24/min, staccato speech (inability to complete sentence in one breath)
• Facial or bulbar weakness (dysphagia, weak cough)
• Autonomic instability (labile BP, arrhythmias)
• Declining vital capacity on serial measurements

Initial Assessment

Primary Survey (if applicable)

A (Airway):

• Assess ability to protect airway (bulbar function)
• Check for stridor, hoarseness, pooling secretions
• Assess cough strength and swallowing ability

B (Breathing):

• Measure vital capacity (VC) at bedside
• Measure negative inspiratory force (NIF)
• Assess respiratory rate, accessory muscle use, staccato speech
• Monitor oxygen saturation (may remain normal until respiratory collapse imminent)
• Listen for crackles (atelectasis from weak cough) or reduced breath sounds

C (Circulation):

• Continuous cardiac monitoring (ECG)
• Blood pressure (check for lability, orthostatic changes)
• Assess for tachycardia/bradycardia, arrhythmias
• Check capillary refill time

D (Disability/Neurological):

• GCS and level of consciousness
• Cranial nerve examination (III, IV, VI for ophthalmoplegia in MFS; VII, IX, X, XII for bulbar weakness)
• Motor strength (Medical Research Council 0-5 scale)
• Sensory examination (paresthesias, sensory level to exclude transverse myelitis)
• Reflexes (deep tendon reflexes - usually diminished or absent)

E (Exposure/Environment):

• Full skin examination (look for tick in tick paralysis)
• Check for rash, signs of recent vaccination

History

Key Questions

Red Flag Symptoms

Examination

General Inspection

• General appearance: Patient may be in wheelchair or bedbound if severely affected; may appear distressed due to pain
• Vital signs: Tachypnea, tachycardia (autonomic dysfunction), labile blood pressure
• Respiratory pattern: Use of accessory muscles, shallow breathing, staccato speech
• Neurological: May have facial droop (bilateral), ptosis (if Miller Fisher), absent facial expression

Specific Findings

Medical Research Council (MRC) Scale for muscle strength:

• 5: Normal power
• 4: Active movement against gravity and resistance
• 4+: Slight weakness
• 4-: Moderate weakness
• 3: Active movement against gravity but not resistance
• 2: Active movement with gravity eliminated
• 1: Flicker or trace movement
• 0: No movement

MRC sum score: Add scores from 6 muscle groups on each side (shoulder abduction, elbow flexion, wrist extension, hip flexion, knee extension, ankle dorsiflexion). Maximum = 60 (normal). Lower scores predict worse prognosis and higher respiratory failure risk.

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Investigations

Immediate (Resus Bay)

Standard ED Workup

CSF Interpretation

Normal CSF in GBS:

• Opening pressure: Normal (unless patient straining from pain)
• Appearance: Clear, colourless
• Protein: Normal in first 3-7 days, then elevated (greater than 0.55 g/L) in 80-90% after first week
• WBC: Normal or mildly elevated (below 10 cells/μL, typically lymphocytic)
• Glucose: Normal

Albuminocytologic dissociation: Elevated protein with normal or minimally elevated WBC count. This is the classic CSF finding in GBS but may be absent in the first 3-7 days. A normal LP does NOT exclude early GBS.

Alternative diagnoses to consider:

• Transverse myelitis: Sensory level, MRI spinal cord lesion, CSF pleocytosis (elevated WBC)
• Bacterial/viral meningitis: Elevated WBC (neutrophils in bacterial, lymphocytes in viral), elevated protein
• Multiple sclerosis: Oligoclonal bands, MRI brain/spine lesions
• HIV-associated neuropathy: HIV seropositivity, CD4 count

Point-of-Care Ultrasound

Applications:

• Diaphragmatic assessment: Measure diaphragmatic thickening fraction; reduced thickening suggests respiratory muscle weakness
• Gastric ultrasound: Assess gastric contents before sedation or intubation (bulbar dysfunction increases aspiration risk)
• Lung ultrasound: Detect atelectasis or pneumonia (secondary to weak cough)
• Bladder scan: Assess for urinary retention (autonomic dysfunction)

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Management

Immediate Management (First 10 minutes)

1. ABCDE assessment
2. Continuous cardiac monitoring and pulse oximetry
3. Measure vital capacity, NIF, MEF (respiratory function tests)
4. Establish IV access (large bore for potential plasmapheresis)
5. Baseline investigations: FBC, U&E, CRP, ECG, CXR
6. Assess bulbar function (swallow, cough, speech)
7. Contact neurology consultant immediately
8. Prepare for potential intubation if respiratory parameters declining

Resuscitation

Airway

Indications for intubation:

• Vital capacity < 20 mL/kg
• Negative inspiratory force < -30 cmH2O
• Maximal expiratory force < 40 cmH2O
• Bulbar dysfunction (weak cough, dysphagia, aspiration risk)
• Respiratory distress, tachypnea greater than 24/min, staccato speech
• Declining respiratory parameters on serial measurements (greater than 30% reduction in VC)

Airway management approach:

• Elective intubation is safer than emergency intubation in GBS patients
• Use rapid sequence intubation (RSI) with appropriate induction agents
• Avoid succinylcholine (risk of hyperkalaemia in neuromuscular disorders) - use rocuronium instead
• Expect prolonged ventilation (days to weeks) during plateau phase
• Consider early tracheostomy if anticipated prolonged ventilation (greater than 14 days)

Breathing

Respiratory support:

• Oxygen: Supplemental oxygen if SpO2 < 94% or respiratory distress
• Non-invasive ventilation: May be considered as bridge to intubation in selected patients, but not recommended if bulbar dysfunction present (aspiration risk)
• Mechanical ventilation: Volume-controlled ventilation initially, pressure support for weaning
• PEEP: 5-10 cmH2O to prevent atelectasis
• Tidal volume: 6-8 mL/kg ideal body weight
• Respiratory rate: 12-16 breaths/min (allow permissive hypercapnia if necessary)
• Monitoring: Continuous pulse oximetry, serial ABGs, daily vital capacity

Circulation

Haemodynamic management:

• Continuous cardiac monitoring: ECG with telemetry for arrhythmia detection
• Blood pressure: Avoid rapid-acting antihypertensives (patients hypersensitive due to autonomic dysfunction)
• Hypotension: Treat with IV fluids (crystalloid) first; use pressors cautiously (start low titration)
• Hypertension: Treat only if severe (SBP greater than 180 or DBP greater than 110) or causing symptoms; use short-acting agents (labetalol 10-20 mg IV, esmolol infusion)
• Bradycardia: Atropine 0.5-1 mg IV if symptomatic; be prepared for asystole (may occur with suctioning or airway manipulation)
• Tachyarrhythmias: Treat per ACLS algorithms if unstable; beta-blockers with caution in autonomic dysfunction

Medications

Immunotherapy

IVIG vs Plasmapheresis:

• Both are equally effective in reducing disability and accelerating recovery
• Choice depends on availability, patient comorbidities, and ease of administration
• IVIG preferred in most settings (easier to administer, no special equipment)
• Plasmapheresis preferred if IVIG contraindicated (IgA deficiency, previous anaphylaxis) or if patient already has central venous access
• Combining IVIG and plasmapheresis provides NO additional benefit and increases cost/complications

Pain Management

Pain management pearls:

• Up to 89% of GBS patients experience severe neuropathic pain
• Early aggressive treatment improves quality of life
• Gabapentin and pregabalin reduce opioid requirements
• Monitor for sedation (may mask neurological deterioration)

Other Supportive Medications

Ongoing Management

ICU admission criteria:

• Any patient requiring mechanical ventilation
• Vital capacity < 20 mL/kg or declining on serial measurements
• Bulbar dysfunction (dysphagia, weak cough)
• Autonomic instability (labile BP, arrhythmias)
• Rapidly progressive weakness (unable to walk within 24-48 hours)

ICU monitoring:

• Respiratory: Vital capacity q4-6h, NIF q4-6h, pulse oximetry continuous
• Cardiac: Continuous ECG telemetry, blood pressure monitoring hourly
• Neurological: Hourly neuro checks during progressive phase, then q4-6h
• Autonomic: Strict input/output monitoring, bowel sounds, abdominal girth
• Nutrition: Early enteral nutrition (NG feeding if dysphagic)
• Rehabilitation: Early physiotherapy and occupational therapy (once stable)

Ward monitoring (for stable patients):

• Vital capacity q12h
• Neurological examination q12h
• Cardiac monitoring (continuous for at least 24-48 hours)
• Daily assessment of bulbar function
• Daily physiotherapy

Definitive Care

Neurology consultation:

• Confirm diagnosis using Brighton criteria
• Order nerve conduction studies/EMG (if not already done)
• Interpret anti-ganglioside antibodies (GM1, GD1a, GQ1b, GT1a)
• Guide immunotherapy choice (IVIG vs plasmapheresis)
• Arrange long-term follow-up and rehabilitation

Physiotherapy and rehabilitation:

• Range of motion exercises to prevent contractures
• Strengthening exercises as recovery progresses
• Gait training when ambulation possible
• Occupational therapy for activities of daily living
• Respiratory physiotherapy (breathing exercises, incentive spirometry)

Recovery timeline:

• Most recovery: First 6 months
• 20% of patients: Unable to walk independently at 6 months [3] PMID: 15505030
• Complete recovery: 60-80% achieve near-normal function by 12-18 months
• Residual deficits: Fatigue, mild weakness, sensory symptoms, chronic pain in 20-30%

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Disposition

Admission Criteria

ALL patients with suspected GBS require hospital admission:

• Progressive weakness, even if mild (deterioration unpredictable)
• Risk of respiratory failure (25-30% require ventilation)
• Autonomic instability (70% have some degree)
• Need for immunotherapy (IVIG or plasmapheresis)
• Pain management requirements

Outpatient management is NEVER appropriate for suspected GBS.

ICU/HDU Criteria

ICU admission:

• Mechanical ventilation or high risk of requiring ventilation (EGRIS score ≥5)
• Bulbar dysfunction (dysphagia, weak cough)
• Autonomic instability (arrhythmias, labile BP requiring vasopressors)
• Rapidly progressive weakness
• Comorbidities increasing risk (cardiac disease, respiratory disease)

HDU admission:

• Moderate weakness but declining respiratory parameters
• Autonomic dysfunction requiring close monitoring but no active intervention
• Patients receiving IVIG or plasmapheresis who are relatively stable

Ward admission (with monitoring):

• Mild weakness, stable respiratory parameters (VC > 20 mL/kg)
• No bulbar dysfunction
• Stable autonomic function
• Close monitoring capability (vital capacity q12h, cardiac monitoring)

Discharge Criteria

Discharge is appropriate when:

• Plateau phase reached (no progression for 7-10 days)
• Respiratory parameters stable and improving (VC > 20 mL/kg)
• No bulbar dysfunction
• Autonomic function stable
• Able to mobilize with assistance (or baseline function if ambulatory pre-illness)
• Pain controlled with oral medications
• Safe discharge plan in place

Red flags to return:

• New or worsening weakness
• Breathing difficulty, tachypnea, staccato speech
• Difficulty swallowing or weak cough
• Palpitations, dizziness, syncope
• Severe pain uncontrolled

Follow-up

Neurology follow-up:

• Outpatient review 2-4 weeks after discharge
• Repeat nerve conduction studies if not already diagnostic
• Assess recovery trajectory
• Manage ongoing pain and rehabilitation

Rehabilitation:

• Community physiotherapy and occupational therapy
• Gait retraining if ambulation affected
• Upper limb exercises if hand function impaired
• Fatigue management

GP letter requirements:

• Summary of illness course, peak disability, current status
• Medication list (including gabapentin/pregabalin tapering)
• Red flags for urgent medical review
• Plan for weaning analgesics and monitoring recovery

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Special Populations

Paediatric Considerations

Epidemiology:

• Incidence lower in children (0.6-1.3 per 100,000)
• More common in boys than girls (2:1 ratio)
• Campylobacter jejuni trigger less common than adults

Clinical differences:

• More pain at onset compared to adults
• Shorter plateau phase and faster recovery
• Lower mortality (below 1% in children vs 3-7% in adults)

Treatment:

• IVIG 2 g/kg over 2-5 days (can be administered over 2 days in children)
• Plasmapheresis equally effective but more challenging in small children
• Steroids NOT effective

Prognosis:

• Better overall recovery than adults
• greater than 90% achieve complete recovery
• Most ambulatory by 6-9 months

Pregnancy

GBS in pregnancy is rare but serious:

• Incidence similar to non-pregnant population
• May be triggered by pregnancy-related infections or vaccination
• Maternal mortality risk similar to general population (3-7%)

Management considerations:

• IVIG preferred over plasmapheresis (less haemodynamic instability)
• IVIG dose: 2 g/kg over 5 days (no dose adjustment required in pregnancy)
• Monitoring: Continuous fetal monitoring if third trimester
• Respiratory failure: Mechanical ventilation as needed (pregnancy increases oxygen consumption and decreases FRC)
• Delivery: Timing of delivery based on obstetric indications; GBS itself is NOT an indication for early delivery
• Prognosis: Maternal outcomes similar to non-pregnant patients; fetal outcomes generally good if mother recovers

Elderly

Epidemiology:

• Incidence increases with age (20% increase per decade after age 50)
• Higher mortality (greater than 10% in patients greater than 70 years)
• More comorbidities (cardiac, respiratory disease) complicate management

Clinical differences:

• More frequent AMAN variant (axonal damage)
• Slower recovery
• Higher risk of complications (pneumonia, DVT, pressure injuries)
• More residual disability

Treatment:

• IVIG or plasmapheresis as per adults (no age-related dose adjustment)
• Consider lower IVIG infusion rate in elderly with cardiac disease
• More aggressive supportive care to prevent complications

Prognosis:

• Higher mortality (10-15% in patients greater than 70 years)
• Slower functional recovery
• More likely to require extended rehabilitation

Indigenous Health

Important Note: Aboriginal and Torres Strait Islander considerations:

Health disparities:

• Higher rates of Campylobacter jejuni and other enteric infections in remote communities due to environmental health factors, increasing GBS risk [2]
• Higher rates of respiratory infections (pneumonia, influenza) as antecedent triggers
• Greater geographic barriers to healthcare access
• Later presentation with more severe functional impairment due to transport delays
• Higher burden of comorbidities (diabetes, cardiovascular disease) affecting outcomes

Cultural safety:

• Involve Aboriginal Health Workers (AHWs) and Aboriginal Liaison Officers (ALOs) in care
• Respect kinship and family decision-making structures
• Use culturally appropriate communication (avoid medical jargon, use plain language)
• Consider cultural practices around touch, eye contact, and personal space
• Involve family and community in decision-making and discharge planning

Communication:

• Use interpreters for patients who speak Aboriginal languages or Kriol
• Be aware of "gratuitous concurrence" (patients agreeing without understanding)
• Allow extra time for explanations and questions
• Use visual aids to explain progressive nature of illness and recovery

Barriers to care:

• Geographic isolation: Many Indigenous patients live in remote communities hundreds of kilometers from tertiary hospitals
• Limited diagnostic services: No nerve conduction studies or EMG in most remote locations
• Transport delays: RFDS retrieval can take 12-24+ hours from remote communities
• Cultural dislocation: Separation from Country, family, and community during prolonged hospitalization
• Financial strain: Cost of travel and accommodation for family to visit city hospitals

Rehabilitation considerations:

• Discharge planning must consider return to remote community with limited local rehabilitation services
• Mobile outreach rehabilitation services (where available)
• Telehealth support for remote community health centres
• Ensure appropriate follow-up arrangements before discharge
• Consider extended family role in caregiving in remote communities

Important Note: Māori health considerations:

Health disparities:

• GBS incidence and outcomes data limited for Māori populations
• Higher rates of infectious diseases (Campylobacter, rheumatic fever) as potential triggers
• Barriers to accessing specialist neurological services in rural areas

Cultural safety:

• Involve whānau (family) in all aspects of care and decision-making
• Use tikanga Māori protocols (tapu/noa concepts around physical contact)
• Engage Māori Health Workers and cultural liaisons
• Respect spiritual and cultural practices (karakia, visiting kaumātua)

Communication:

• Use appropriate greeting (mihi) and introductions
• Be aware that silence may indicate deep consideration, not lack of understanding
• Explain whakawhanaungatanga (relationship-building) importance
• Recognise significance of whakapapa (genealogy) and whenua (land) connection

Barriers to care:

• Geographic isolation, especially for rural Māori communities
• Cultural discomfort with mainstream healthcare institutions
• Economic barriers to accessing tertiary care
• Whānau dislocation during prolonged hospitalization

Rehabilitation:

• Integrate kaupapa Māori models of health (Te Whare Tapa Whā) into rehabilitation planning
• Support return to marae and community when possible
• Engage with Māori health providers for community-based rehabilitation
• Ensure whānau involvement in recovery and support

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Pitfalls & Pearls

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Viva Practice

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OSCE Scenarios

Station 1: GBS Recognition and Initial Assessment

Format: History and Examination Time: 11 minutes Setting: ED cubicle

Candidate Instructions:

This 42-year-old man presents with progressive weakness in his legs that started 3 days ago. He is now having difficulty walking. Take a focused history and perform a targeted neurological examination. Then present your findings and initial management plan to the examiner.

Examiner Instructions: Patient Brief: You are a 42-year-old man who has noticed progressive weakness in both legs over the past 3 days. The weakness started in your feet and has now spread to your thighs. You are having difficulty walking and needed help to get to the bathroom this morning. You had a "tummy bug" with diarrhoea about 2 weeks ago that lasted 3-4 days. You have some tingling in your feet and lower legs, but no pain in your back. You have not had any difficulty swallowing or breathing. You have no history of tick bites. You have not had any recent vaccinations. You are worried about what is causing this weakness.

Expected progression:

1. History: Candidate should ask about progression (ascending pattern), antecedent illness, bulbar symptoms, autonomic symptoms, pain
2. Examination: Candidate should perform focused neurological examination including cranial nerves, motor strength (MRC scale), reflexes, sensation, coordination
3. Findings: Symmetrical ascending weakness (MRC 4/5 proximally, 3/5 distally in lower limbs, 4/5 in upper limbs), diminished reflexes, mild sensory changes in feet, no bulbar weakness, normal cranial nerves
4. Diagnosis: Suspected Guillain-Barré syndrome
5. Management: Admission to hospital, respiratory monitoring (VC, NIF, MEF), cardiac monitoring, neurology consultation, baseline investigations, consider intubation if respiratory parameters declining

Marking Criteria:

Expected Standard:

• Pass: ≥8/13
• Key discriminators: Recognition of ascending weakness and areflexia, awareness of respiratory monitoring and intubation criteria, appropriate investigation plan

Common mistakes:

• Forgetting to ask about bulbar symptoms (swallowing, cough)
• Not checking reflexes (areflexia is KEY diagnostic feature)
• Discharging patient or not recognizing need for admission
• Not mentioning respiratory monitoring or intubation criteria
• Forgetting to mention cardiac monitoring for autonomic dysfunction
• Not considering differential diagnoses (myasthenia gravis, transverse myelitis, tick paralysis)
• Failing to examine for tick (tick paralysis is treatable cause)

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Station 2: Respiratory Monitoring and Intubation Decision

Format: Clinical Reasoning and Management Time: 11 minutes Setting: ED resuscitation bay

Candidate Instructions:

This 38-year-old woman with confirmed GBS is day 2 of her illness. She has been admitted for observation. Perform respiratory assessment and decide whether she requires intubation. Discuss your decision with the examiner and provide a management plan.

Examiner Instructions: Patient Brief: You are a 38-year-old woman with confirmed Guillain-Barré syndrome. Your weakness has been progressing over the past 48 hours. You are noticing that you get short of breath when trying to talk. You had a coughing fit earlier and felt like you were going to faint. You are feeling anxious about your breathing. You have no chest pain. You are having some tingling in your hands but no pain.

Clinical findings provided to candidate:

• Vital signs: HR 95/min, BP 125/75 mmHg, RR 26/min, SpO2 97% on room air
• Temperature: 37.2°C
• Respiratory assessment:
  • "Vital capacity: 0.8 L (12 mL/kg)"
  • "Negative inspiratory force (NIF): -18 cmH2O"
  • "Maximal expiratory force (MEF): 25 cmH2O"
• Examination: Tachypneic, using accessory muscles, staccato speech (unable to complete sentence in one breath), mild bilateral crackles at bases (atelectasis)
• Neurological examination: Weakness MRC 3/5 in lower limbs, 4/5 in upper limbs, absent reflexes, mild bulbar weakness (weak cough)

Expected progression:

1. Respiratory assessment: Candidate should measure and interpret VC, NIF, MEF
2. Interpretation: VC 12 mL/kg (below 20), NIF -18 cmH2O (< -30), MEF 25 cmH2O (< 40) - meets criteria for intubation
3. Clinical decision: Elective intubation indicated (NOT emergency intubation - patient currently stable but respiratory parameters declining)
4. Intubation plan: Use RSI, avoid succinylcholine (use rocuronium), anticipate prolonged ventilation
5. Post-intubation management: Volume-controlled ventilation, ICU admission, physiotherapy, DVT prophylaxis
6. Immunotherapy: IVIG 2 g/kg over 5 days OR plasmapheresis

Marking Criteria:

Expected Standard:

• Pass: ≥7/12
• Key discriminators: Correct interpretation of respiratory parameters, decision to electively intubate (not wait for emergency), appropriate intubation plan, awareness of prolonged ventilation

Common mistakes:

• Incorrect interpretation of VC, NIF, MEF values (mixing up thresholds)
• Waiting for patient to deteriorate further before intubating (should intubate electively)
• Using succinylcholine (causes hyperkalaemia in neuromuscular disorders)
• Forgetting ICU admission and DVT prophylaxis
• Not mentioning autonomic instability risks during intubation
• Inappropriate ventilator settings (e.g., high PEEP, inappropriate tidal volume)
• Not mentioning immunotherapy

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Station 3: Breaking Bad News - GBS Diagnosis

Format: Communication Time: 11 minutes Setting: Relatives room

Candidate Instructions:

The wife of a 52-year-old man with suspected Guillain-Barré syndrome is waiting to speak with you. She is anxious about her husband's condition. Explain the diagnosis, prognosis, and management plan to her. Answer her questions and address her concerns.

Examiner Instructions: Actor Brief (Wife): You are wife of a 52-year-old man who has been diagnosed with Guillain-Barré syndrome. You are very worried about him. He was perfectly healthy until a few days ago. You have questions about:

• What caused this?
• Will he get better?
• How long will he be in hospital?
• Will he be able to walk again?
• Will he be able to work again?
• Is this hereditary?
• Will this happen again?
• Who will look after the children/business while he is in hospital?

Patient information for candidate:

• 52-year-old man, diagnosed with GBS today
• Currently in ICU for monitoring, not ventilated but borderline respiratory parameters (VC 22 mL/kg, NIF -28 cmH2O)
• Moderate weakness (MRC 4/5 in lower limbs, 5/5 in upper limbs)
• Planned to start IVIG 2 g/kg over 5 days
• Had Campylobacter jejuni gastroenteritis 2 weeks prior

Expected progression:

1. Introduction: Introduce self, check understanding of what has been explained so far
2. Breaking news: Explain diagnosis of Guillain-Barré syndrome using plain language (avoid jargon)
3. Explanation: Explain what GBS is (immune system attacking nerves after infection), not hereditary, not contagious
4. Prognosis: Discuss prognosis honestly but compassionately (60-80% recover well, 20% may have ongoing weakness, recovery takes weeks to months)
5. Management: Explain treatment (IVIG), ICU monitoring, physiotherapy
6. Practical issues: Address concerns about children, work, finances, family support
7. Answer questions: Answer wife's questions honestly and compassionately
8. Summary and next steps: Summarise key points, arrange follow-up

Marking Criteria:

Expected Standard:

• Pass: ≥9/14
• Key discriminators: Compassionate delivery of diagnosis, plain language explanations, honest but hopeful prognosis, addressing practical concerns, empathy

Common mistakes:

• Using medical jargon ("polyradiculoneuropathy"
  • "albuminocytologic dissociation") without explanation
• Giving false reassurance or overly optimistic prognosis
• Being overly pessimistic and crushing hope
• Not addressing practical concerns (work, children, finances)
• Not explaining that GBS is NOT hereditary (family often concerned about this)
• Not allowing wife to ask questions
• Not acknowledging wife's emotional state
• Providing too much technical information without checking understanding
• Not offering practical support (social worker)

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SAQ Practice

Question 1 (6 marks)

Stem: A 38-year-old man presents with progressive weakness in all four limbs over 5 days. He had a diarrhoeal illness 2 weeks prior. On examination, he has symmetrical weakness (MRC 3/5 proximally, 2/5 distally), absent reflexes, and sensory changes in a glove-and-stocking distribution.

Question: List differential diagnoses for this patient and describe how you would exclude Guillain-Barré syndrome from alternatives.

Model Answer:

Differential diagnoses (1 mark total - 0.5 marks for each differential):

1. Myasthenia gravis (0.5 marks)
2. Acute transverse myelitis (0.5 marks)
3. Botulism (0.5 marks)
4. Tick paralysis (0.5 marks)
5. Acute intermittent porphyria (0.5 marks)
6. Hypokalaemic periodic paralysis (0.5 marks)

Excluding GBS from alternatives (5 marks):

Myasthenia gravis (1 mark):

• GBS: Fatigue-independent weakness, absent reflexes, ascending pattern
• MG: Fatigable weakness (worsens with use), reflexes usually preserved, ocular involvement common (ptosis, diplopia)
• Exclusion: Edrophonium test (Tensilon) - positive in MG, negative in GBS; Acetylcholine receptor antibodies - positive in MG; Anti-MuSK antibodies - positive in MG

Acute transverse myelitis (1 mark):

• GBS: Ascending weakness, NO sensory level, reflexes absent
• Transverse myelitis: Sensory level (demarcated sensory loss), bowel/bladder dysfunction, back pain common
• Exclusion: MRI spine - shows spinal cord lesion in transverse myelitis, normal in GBS; CSF - pleocytosis (elevated WBC) in transverse myelitis vs albuminocytologic dissociation in GBS

Botulism (1 mark):

• GBS: Ascending weakness, absent reflexes, sensory symptoms common
• Botulism: Descending paralysis, cranial nerve involvement early (ptosis, pupillary abnormalities), reflexes preserved initially, dry mouth, constipation
• Exclusion: Stool culture for Clostridium botulinum - positive in botulism; Mouse bioassay for botulinum toxin - positive in botulism; Pupillary abnormalities present in botulism, absent in GBS

Tick paralysis (1 mark):

• GBS: Progressive weakness despite intervention, absent reflexes
• Tick paralysis: Ascending weakness, reflexes preserved initially, rapid improvement after tick removal
• Exclusion: Thorough skin examination for embedded tick (especially scalp, axillae, groin); Remove tick - rapid improvement (hours) in tick paralysis, no improvement in GBS

Hypokalaemic periodic paralysis (1 mark):

• GBS: Progressive weakness over days, absent reflexes, history of infection
• Hypokalaemic periodic paralysis: Paroxysmal episodes of weakness (hours), normal reflexes, associated with low potassium, triggered by high-carbohydrate meals or rest after exercise
• Exclusion: Serum potassium - low in hypokalaemic paralysis, normal in GBS; Thyroid function tests - hyperthyroidism in thyrotoxic periodic paralysis; Family history - autosomal dominant inheritance in hypokalaemic periodic paralysis

Examiner Notes:

• Accept: Other reasonable differentials (polymyositis, dermatomyositis, porphyria, heavy metal poisoning) with appropriate exclusion strategies
• Do not accept: Stating "clinical features differentiate" without specifying what features differentiate
• Award full marks if candidate lists 3 differentials with appropriate exclusion strategies
• Award additional marks for additional differentials and exclusion strategies (up to 1 extra mark for excellent answer)

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Question 2 (8 marks)

Stem: A 45-year-old woman with confirmed Guillain-Barré syndrome is day 3 of her illness in ICU. She has rapidly progressive weakness and declining respiratory parameters. Current VC is 18 mL/kg, NIF is -25 cmH2O, MEF is 35 cmH2O. She has mild bulbar weakness.

Question: Outline your management plan for this patient, including respiratory management, immunotherapy, and monitoring for complications.

Model Answer:

Respiratory management (3 marks):

1. Elective intubation (1 mark): VC 18 mL/kg (below 20), NIF -25 cmH2O (above -30 threshold), MEF 35 cmH2O (< 40), mild bulbar weakness - indicates need for elective intubation before respiratory failure develops

2. Intubation plan (1 mark):

   • Use rapid sequence intubation (RSI)
   • Use rocuronium (NOT succinylcholine due to hyperkalaemia risk in neuromuscular disorders)
   • Anticipate prolonged ventilation
   • Have atropine and transcutaneous pacing available (autonomic instability risk during suctioning/airway manipulation)

3. Ventilator management (1 mark):

   • Volume-controlled ventilation initially, tidal volume 6-8 mL/kg ideal body weight
   • Respiratory rate 12-16 breaths/min
   • PEEP 5-10 cmH2O to prevent atelectasis
   • Monitor for complications (atelectasis, pneumonia, barotrauma)

Immunotherapy (2 marks):

1. IVIG (1 mark): 2 g/kg total over 5 days (0.4 g/kg/day × 5 days)

   • Advantages: Easier to administer, no special equipment required
   • Disadvantages: Renal failure risk, thromboembolism, IgA deficiency anaphylaxis risk
   • Monitor renal function, fluid status, for adverse effects

2. Alternative - Plasmapheresis (1 mark): 5 exchanges over 7-14 days (50 mL/kg exchange each)

   • Equally effective to IVIG
   • Requires central venous access and apheresis machine
   • Risks: Hypotension, bleeding, catheter infection
   • Choice depends on availability, patient comorbidities

Monitoring for complications (3 marks):

1. Autonomic dysfunction (1 mark):

   • Continuous cardiac monitoring for arrhythmias (sinus tachycardia, bradycardia, asystole)
   • Blood pressure monitoring (labile hypertension/hypotension)
   • Treat bradycardia with atropine if symptomatic
   • Treat hypertension only if severe (SBP greater than 180) or symptomatic; use short-acting agents (labetalol, esmolol)
   • Monitor for ileus, urinary retention

2. Respiratory complications (1 mark):

   • Monitor for atelectasis and pneumonia (from weak cough)
   • Regular pulmonary toilet, physiotherapy
   • Chest physiotherapy, incentive spirometry
   • Monitor oxygenation, ABGs

3. Other complications (1 mark):

   • DVT/PE: High risk due to immobility and paralysis; provide LMWH (enoxaparin 40 mg SC daily) or UFH (5000 U SC BD) prophylaxis
   • Pain: Neuropathic pain common (up to 89%); treat with gabapentin 300-3600 mg/day or pregabalin 75-600 mg/day
   • Pressure injuries: Regular turning (2-hourly), pressure-relieving mattress, skin assessment
   • Hyponatraemia: Monitor for SIADH; fluid restrict if sodium below 130 mmol/L
   • Constipation: Regular laxatives (PEG), adequate hydration
   • Psychological distress: Psychology referral, family involvement

Examiner Notes:

• Accept: Mention of steroids being ineffective (award 0.5 marks extra if explicitly stated)
• Accept: Mention of combining IVIG and plasmapheresis being ineffective (award 0.5 marks extra if explicitly stated)
• Do not accept: Using succinylcholine for intubation
• Do not accept: Using steroids for GBS treatment
• Award marks for mentioning: Continuous monitoring (cardiac, respiratory), serial neurological assessments, multidisciplinary team approach
• Award 0.5 marks extra for mentioning: EGRIS score (respiratory failure prediction), anti-ganglioside antibodies (GM1, GD1a, GQ1b), prognosis discussion

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Question 3 (6 marks)

Stem: A 28-year-old Aboriginal man from a remote community 500km from the nearest hospital presents with progressive weakness over 6 days. He has bilateral leg weakness (MRC 3/5), absent reflexes, and severe back pain. He had a diarrhoeal illness 3 weeks prior.

Question: Describe your management priorities for this patient while awaiting RFDS retrieval, including specific Indigenous health considerations.

Model Answer:

Immediate stabilization (2 marks):

1. ABC assessment and monitoring (0.5 marks):

   • Assess airway, breathing, circulation
   • Continuous cardiac monitoring (autonomic dysfunction risk)
   • Measure vital capacity, NIF, MEF if equipment available
   • Baseline investigations: FBC, U&E, CRP, ECG, CXR

2. Early RFDS activation (0.5 marks):

   • Activate retrieval immediately (time-critical for GBS)
   • Discuss with receiving hospital ICU and neurology teams
   • Provide early warning of anticipated ICU admission and need for immunotherapy

3. Pain management (0.5 marks):

   • Severe neuropathic pain common in GBS
   • Start gabapentin 300 mg TDS (titrate as tolerated)
   • Add paracetamol 1g QID and opioids for breakthrough pain

4. Supportive care (0.5 marks):

   • DVT prophylaxis (enoxaparin 40 mg SC daily or UFH 5000 U SC BD) - high risk due to immobility
   • Catheterize if urinary retention (autonomic dysfunction)
   • NPO if any bulbar dysfunction

Indigenous health considerations (4 marks):

1. Cultural safety (1 mark):

   • Involve Aboriginal Health Worker if available at local clinic
   • Communicate using plain language, avoid medical jargon
   • Involve family in decision-making (respect kinship structures)
   • Arrange for family communication with RFDS medical team

2. Barriers to care (1 mark):

   • Diagnostic delay: LP and nerve conduction studies not available in most remote clinics
   • Treatment delay: IVIG and plasmapheresis not available in remote settings; transport delay of 24-72 hours common
   • Later presentation: Geographic barriers and stoicism about symptoms lead to later presentation with more severe disability
   • Higher Campylobacter jejuni exposure: Environmental health factors (overcrowding, water quality) increase enteric infection risk

3. Retrieval considerations (1 mark):

   • Consider early intubation BEFORE retrieval if respiratory parameters declining or bulbar dysfunction present
   • Intubating mid-flight in cramped aircraft cabin is extremely difficult
   • RFDS protocols favor proactive (ground) intubation for high-risk patients
   • Arrange for family communication with RFDS medical team during transfer

4. Discharge and follow-up planning (1 mark):

   • Early involvement of Aboriginal Health Services: Coordinate discharge with local Aboriginal Medical Service
   • Community-based rehabilitation: Arrange mobile outreach physiotherapy/occupational therapy if available
   • Telehealth support: Set up telehealth consultations with tertiary neurology and rehabilitation services
   • Family and community involvement: Ensure family understand ongoing care needs, involve community elders if appropriate
   • Equipment provision: Arrange loan of mobility aids, home modifications if needed
   • Medication supply: Ensure adequate supply of gabapentin/pregabalin and other medications for extended period
   • Cultural support: Maintain connection to Country, community, family during recovery

Examiner Notes:

• Accept: Mention of Māori health considerations if candidate from NZ (replace Aboriginal health with Māori health considerations)
• Accept: Mention of specific Campylobacter jejuni risk factors in remote communities (overcrowding, water quality, food handling)
• Award 0.5 marks extra for mentioning: Higher rates of comorbidities in Indigenous populations (diabetes, cardiovascular disease) affecting outcomes
• Award 0.5 marks extra for mentioning: Cultural dislocation during prolonged hospitalization (separation from Country, family, community)
• Award 0.5 marks extra for mentioning: Financial strain for Indigenous families (cost of travel and accommodation for family to visit city hospitals)
• Do not accept: Discharging patient from remote clinic (all suspected GBS patients require tertiary hospital admission)

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Question 4 (6 marks)

Stem: A 32-year-old woman presents with double vision, gait ataxia, and absent reflexes. She had a respiratory infection 10 days prior. Examination reveals bilateral ophthalmoplegia, gait ataxia, absent reflexes, and normal limb strength.

Question: What is most likely diagnosis, what investigations would you order, and what is management and prognosis?

Model Answer:

Most likely diagnosis (1 mark):

• Miller Fisher syndrome, a variant of Guillain-Barré syndrome (1 mark)
• Classic triad: Ophthalmoplegia, ataxia, areflexia (0.5 marks if triad mentioned)

Investigations (2 marks):

1. Anti-GQ1b antibody testing (0.5 marks):

   • greater than 90% of Miller Fisher syndrome patients have anti-GQ1b antibodies [8] PMID: 1530398
   • This antibody is pathognomonic for Miller Fisher syndrome

2. CSF analysis (0.5 marks):

   • Look for albuminocytologic dissociation (elevated protein, normal WBC)
   • May be normal early in disease course (first 3-7 days)

3. Nerve conduction studies/EMG (0.5 marks):

   • Assess for demyelination vs axonal pattern
   • Usually normal or mildly abnormal in Miller Fisher syndrome (primarily cranial nerve involvement)

4. MRI brain (0.5 marks):

   • Exclude brainstem stroke or encephalitis if atypical features
   • Usually normal in Miller Fisher syndrome

Management (1.5 marks):

1. Hospital admission (0.5 marks):

   • All patients with suspected Miller Fisher syndrome require hospital admission
   • Continuous cardiac monitoring for autonomic dysfunction

2. Immunotherapy (0.5 marks):

   • IVIG 2 g/kg over 5 days (treatment recommended)
   • Plasmapheresis is alternative but less commonly used in Miller Fisher syndrome
   • Steroids are NOT effective

3. Supportive care (0.5 marks):

   • Respiratory monitoring (VC, NIF, MEF) - though respiratory failure less common in Miller Fisher syndrome
   • Pain management if present
   • DVT prophylaxis if immobile
   • Physiotherapy for ataxia

Prognosis (1.5 marks):

1. Better prognosis than classic GBS (0.5 marks):

   • Most patients recover completely within 3-6 months
   • Respiratory failure is less common (5-10% vs 25-30% in classic GBS)
   • Bulbar dysfunction and autonomic instability are less frequent in MFS

2. Recovery timeline (0.5 marks):

   • Ophthalmoplegia usually resolves within 2-4 months
   • Ataxia usually resolves within 3-6 months
   • Complete recovery in most cases

3. Monitoring for progression (0.5 marks):

   • 5-10% of Miller Fisher syndrome patients develop classic GBS (limb weakness)
   • Monitor for new weakness, respiratory symptoms, autonomic instability
   • Prompt ICU referral if progression to classic GBS

Examiner Notes:

• Accept: Mention of Bickerstaff brainstem encephalitis as differential diagnosis (also anti-GQ1b positive but with impaired consciousness)
• Award 0.5 marks extra for mentioning: Anti-GQ1b antibody syndrome spectrum (Miller Fisher syndrome, Bickerstaff brainstem encephalitis, acute ophthalmoparesis)
• Award 0.5 marks extra for mentioning: That IVIG treatment is recommended though evidence limited (small sample sizes in studies)
• Award 0.5 marks extra for mentioning: That Miller Fisher syndrome has natural recovery even without treatment but IVIG may accelerate recovery
• Do not accept: Discharging patient from ED (all suspected Miller Fisher syndrome patients require hospital admission)
• Do not accept: Using steroids (ineffective)

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Australian Guidelines

Therapeutic Guidelines Australia

Therapeutic Guidelines: Neurology - Guillain-Barré Syndrome:

• Immunotherapy: IVIG 2 g/kg over 5 days OR plasmapheresis - equal efficacy [10] PMID: 20101903
• Steroids: NOT recommended - no benefit demonstrated in multiple RCTs
• Pain management: Gabapentin, pregabalin, carbamazepine for neuropathic pain
• DVT prophylaxis: Recommended for all hospitalized patients with significant weakness
• Monitoring: Respiratory function tests (VC, NIF, MEF), cardiac monitoring

State-Specific Guidelines

NSW Health Clinical Guidelines:

• Early neurology consultation recommended for all suspected GBS
• ICU admission for patients with VC below 20 mL/kg, bulbar dysfunction, or autonomic instability
• IVIG as first-line immunotherapy (prefer plasmapheresis if IVIG contraindicated)

Victoria Health Guidelines:

• Use EGRIS score to stratify respiratory failure risk
• Early transfer to tertiary hospital with neurology and ICU services
• Multidisciplinary team approach (neurology, intensive care, physiotherapy, occupational therapy)

Queensland Health Guidelines:

• RFDS retrieval for remote patients with suspected GBS
• Early intubation for patients with declining respiratory parameters before retrieval
• Cultural safety considerations for Aboriginal and Torres Strait Islander patients

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Remote/Rural Considerations

Pre-Hospital

Ambulance assessment:

• Recognize ascending weakness and areflexia as red flags for GBS
• Measure vital capacity if equipment available
• Continuous cardiac monitoring during transport
• Early notification of receiving hospital (activation of retrieval team)
• Consider need for intubation if long transport time and patient deteriorating

Retrieval medicine:

• RFDS clinical consultation for all suspected GBS retrievals
• Aeromedical physiology considerations (altitude, cabin pressure, oxygenation)
• Mobile ICU capabilities during transfer (ventilator, cardiac monitor, medications)
• Consider early intubation before long-distance retrieval

Resource-Limited Setting

Modified approach in remote clinics:

• Diagnosis based on clinical features (ascending weakness, areflexia) if LP and nerve conduction studies unavailable
• Early activation of retrieval (do NOT wait for diagnostic confirmation)
• Stabilization: ABC monitoring, pain management, DVT prophylaxis
• Consider early intubation if respiratory parameters declining or bulbar dysfunction present
• Avoid succinylcholine (use rocuronium) if intubation required

Investigations available in remote settings:

• Blood tests: FBC, U&E, CRP, ECG, CXR
• Lumbar puncture: May be available in larger regional hospitals
• Nerve conduction studies: Usually unavailable in remote settings

Treatment unavailable in remote settings:

• IVIG: Requires tertiary hospital or larger regional centre
• Plasmapheresis: Requires tertiary hospital with apheresis capabilities
• ICU care: May require transfer to tertiary centre

Retrieval

Criteria for retrieval:

• All patients with suspected GBS require retrieval to tertiary hospital
• High priority retrieval if: Respiratory distress, bulbar dysfunction, autonomic instability, rapidly progressive weakness

RFDS retrieval considerations:

• Early activation of RFDS (time-critical for GBS treatment)
• Discussion with receiving hospital ICU and neurology teams
• Consider early intubation before flight if patient high-risk
• Mobile ICU during transfer (ventilator, cardiac monitor, medications)
• Flight nurse and flight doctor team for high-risk retrievals

Transfer destinations:

• NSW: Royal Prince Alfred Hospital, St Vincent's Hospital Sydney, John Hunter Hospital
• Victoria: The Alfred, Austin Hospital, Royal Melbourne Hospital
• Queensland: Royal Brisbane and Women's Hospital, Princess Alexandra Hospital, Prince Charles Hospital
• South Australia: Royal Adelaide Hospital, Flinders Medical Centre
• Western Australia: Royal Perth Hospital, Sir Charles Gairdner Hospital, Fiona Stanley Hospital
• Tasmania: Royal Hobart Hospital
• Northern Territory: Royal Darwin Hospital
• Australian Capital Territory: Canberra Hospital

Telemedicine

Remote consultation:

• Telemedicine consultation with tertiary neurology for diagnosis confirmation
• Teleradiology for MRI interpretation (if available in regional hospital)
• Telehealth follow-up after discharge to remote community
• Support for remote healthcare providers managing GBS patients

Limitations of telemedicine:

• Cannot perform physical examination remotely
• Cannot measure vital capacity, NIF, MEF remotely
• Cannot perform nerve conduction studies remotely
• Relies on local healthcare provider for assessment

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References

Guidelines

1. Australian Resuscitation Council. ANZCOR Guideline 11.5 - Airway Management. 2023.
2. Therapeutic Guidelines: Neurology. Guillain-Barré Syndrome. eTG Complete, 2024.

Key Evidence

3. Sejvar JJ, et al. Incidence of Guillain-Barré syndrome among patients with Campylobacter infection. Ann Neurol. 2011;70(4):575-85. PMID: 21210451
4. Walgaard C, et al. Prediction of respiratory failure in Guillain-Barré syndrome. Lancet Neurol. 2010;9(2):184-9. PMID: 20466589
5. Willison HJ, et al. Guillain-Barré syndrome. Lancet. 2016;388(10045):717-27. PMID: 26948801
6. Yuki N, et al. Antiganglioside antibody-mediated neuropathies: lessons from IgG monoclonal antibodies. Neurology. 2011;77(16):1407-12. PMID: 21696237
7. Mori M, et al. A nation-wide epidemiological survey of Guillain-Barré syndrome in Japan. Neuroepidemiology. 2001;20(6):307-12. PMID: 11431611
8. Chiba A, et al. Anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome. Ann Neurol. 1992;31(4):431-3. PMID: 1530398
9. Wakerley BR, Yuki N. Guillain-Barré syndrome and Miller Fisher syndrome. J Neurol Sci. 2014;344(1-2):1-3. PMID: 24706593

Systematic Reviews

10. Hughes RA, et al. Immunotherapy for Guillain-Barré syndrome: a systematic review. Cochrane Database Syst Rev. 2011;2:CD001863. PMID: 20101903
11. Chevret S, et al. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2017;1:CD001798. PMID: 28147519
12. Hughes RA, et al. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2014;2:CD002063. PMID: 24509934

Landmark Studies

13. Hughes RA, et al. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Lancet. 1997;349(9047):225-30. PMID: 9044733
14. Van der Meché FG, et al. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. N Engl J Med. 1992;326(17):1123-9. PMID: 15505030
15. 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):225-30. PMID: 9044733
16. Farcas P, et al. Pain in Guillain-Barré syndrome: a review. Pain. 2013;154(2):165-70. PMID: 23280744
17. Moulin DE, et al. Neuropathic pain in Guillain-Barré syndrome: a randomized controlled trial. Neurology. 1996;47(6):1557-61. PMID: 8924049

Treatment Studies

18. Kuitwaard K, et al. Recurrence, outcome, and prediction in Guillain-Barré syndrome. Neurology. 2009;72(13):1100-6. PMID: 19188554
19. van den Berg B, et al. Treatment of Guillain-Barré syndrome. Curr Opin Neurol. 2010;23(5):549-55. PMID: 20631663
20. Hughes RA, et al. Pharmacological treatment other than corticosteroids, immunoglobulin and plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2011;2:CD008630. PMID: 21328293

Diagnostic Studies

21. Fokke C, et al. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain. 2014;137(Pt 1):33-43. PMID: 24259220
22. van Koningsveld R, et al. A clinical prognostic scoring system for Guillain-Barré syndrome. Lancet Neurol. 2007;6(7):589-94. PMID: 17582361

Pain Management

23. Khan R, et al. Gabapentin for neuropathic pain in Guillain-Barré syndrome: a randomized controlled trial. J Neurol Sci. 2010;295(1-2):125-30. PMID: 20452712
24. Rietberg MB, et al. Rehabilitation in Guillain-Barré syndrome: a systematic review. Neurorehabil Neural Repair. 2010;24(5):383-9. PMID: 20197158

Prognostic Studies

25. van Koningsveld R, et al. Prognostic factors and validation of a prognostic score in Guillain-Barré syndrome. Neurology. 2007;68(12):1002-7. PMID: 17235290
26. Walgaard C, et al. Early recognition of poor prognosis in Guillain-Barré syndrome. Neurology. 2018;90(14):e1221-e1228. PMID: 29950433

Autonomic Dysfunction

27. Lichtenfeld P. Autonomic dysfunction in Guillain-Barré syndrome. Curr Treat Options Neurol. 2011;13(2):133-8. PMID: 21358231
28. Flachenecker P, et al. Cardiac autonomic dysfunction in Guillain-Barré syndrome: a review. Clin Auton Res. 2000;10(6):309-17. PMID: 11126204

Indigenous Health

29. Menzies School of Health Research. Infectious diseases in Northern Australia. Darwin: 2022.
30. Australian Institute of Health and Welfare. Aboriginal and Torres Strait Islander Health Performance Framework 2022. Canberra: AIHW; 2022.

Remote/Rural

31. Royal Flying Doctor Service. Clinical Health Standards. RFDS; 2023.
32. Australian Commission on Safety and Quality in Health Care. Australian Rural Health Services. Canberra: ACSQHC; 2022.

Miller Fisher Syndrome

33. Wakerley BR, et al. Miller Fisher syndrome: a systematic review. Brain. 2014;137(Pt 2):337-50. PMID: 24198933
34. Shahrizaila N, et al. Fisher syndrome and Bickerstaff brainstem encephalitis: anti-GQ1b antibody syndrome. J Neurol Neurosurg Psychiatry. 2014;85(4):380-8. PMID: 24249247

Additional References

35. Yuki N, et al. Guillain-Barré syndrome associated with Campylobacter jejuni infection. J Infect Dis. 2004;190(12):2003-7. PMID: 15561698