What is the recommended acute management for Guillain‑Barré syndrome, including immunotherapy and supportive care?

Management of Guillain-Barré Syndrome

Immediate Assessment and Stabilization

All patients with suspected GBS require immediate assessment of respiratory function and autonomic stability, as these determine mortality risk and need for ICU-level care. 1

Critical Initial Evaluation

• Measure vital capacity, negative inspiratory force (NIF), and maximum inspiratory/expiratory pressures at presentation and serially 1
• Apply the "20/30/40 rule": patient is at risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1
• Single breath count ≤19 predicts need for mechanical ventilation 1
• Perform continuous cardiac monitoring for arrhythmias and blood pressure instability 1
• Approximately 20% of patients develop respiratory failure requiring mechanical ventilation, which can occur rapidly and sometimes without obvious dyspnea 1

Neurological Examination Specifics

• Grade muscle strength using Medical Research Council scale in neck, arms, and legs 1
• Assess functional disability using GBS disability scale 1
• Test swallowing and coughing ability to identify aspiration risk 1
• Assess for facial weakness, ophthalmoplegia, and check corneal reflex in patients with facial palsy 1

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Diagnostic Workup

Do not delay treatment waiting for diagnostic confirmation if GBS is clinically suspected. 1

Essential Diagnostic Tests

• Neurology consultation for all suspected cases 2, 1
• Cerebrospinal fluid analysis: Look for albumino-cytological dissociation (elevated protein with normal cell count), though do not dismiss GBS based on normal CSF protein in the first week 1, 3
• Electrodiagnostic studies (nerve conduction studies and EMG) to evaluate polyneuropathy and classify subtype (AIDP, AMAN, or AMSAN) 1
• MRI of spine with contrast to rule out compressive lesions and evaluate for nerve root enhancement/thickening 2, 1
• Serum antiganglioside antibody tests for GBS subtypes (e.g., anti-GQ1b for Miller Fisher variant) 2, 1

Laboratory Screening

• Complete blood count, glucose, electrolytes, kidney function, liver enzymes 1
• Serum creatine kinase (CK) - elevation suggests muscle involvement 1
• Screen for reversible causes: HbA1c, vitamin B12, TSH, vitamin B6, folate 1

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First-Line Immunotherapy

Initiate treatment immediately in patients unable to walk unaided within 2-4 weeks of symptom onset. 1, 4, 3

Treatment Options (Equally Effective)

IVIg is generally preferred as first-line therapy because it is easier to administer, more widely available, and has higher completion rates compared to plasma exchange. 4, 3

Intravenous Immunoglobulin (IVIg)

• Dose: 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) 2, 1, 4, 3
• Use ideal body weight for dosing in obese patients, as IVIG distributes in plasma and extracellular fluid spaces that correlate with lean body mass 4
• Check serum IgA levels before first infusion - IgA deficiency increases anaphylaxis risk; use preparations with reduced IgA levels if deficiency confirmed 4

Plasma Exchange (PE)

• Dose: 200-250 ml/kg over 4-5 sessions within 4 weeks of symptom onset 1, 4, 3
• Equally effective as IVIg but requires more specialized equipment and monitoring 4, 5

What NOT to Do

• Do NOT use corticosteroids alone - randomized controlled trials show no benefit and oral corticosteroids may worsen outcomes 4, 5, 3
• Do NOT combine PE followed immediately by IVIg - no additional benefit 5, 3
• Do NOT give a second IVIg course routinely to patients with poor prognosis - evidence does not support this 3

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Admission and Monitoring Criteria

Grade 2 (Moderate Symptoms)

• Some interference with activities of daily living, symptoms concerning to patient 2
• Discontinue immune checkpoint inhibitors if applicable 2
• Neurology consultation and close monitoring required 2, 1

Grade 3-4 (Severe Disease)

Admit to inpatient unit with capability for rapid transfer to ICU-level monitoring 2, 1

Indications include:

• Severe weakness limiting self-care 2, 1
• Any dysphagia, facial weakness, or respiratory muscle weakness 2, 1
• Rapidly progressive symptoms 2, 1

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Supportive Care and Complication Management

Respiratory Management

• Frequent pulmonary function assessment with serial vital capacity and NIF measurements 2, 1
• Daily neurologic evaluation 2, 1

Pain Management

Use gabapentinoids (gabapentin, pregabalin) or duloxetine for neuropathic pain - these can be initiated alongside IVIg without interaction or delay 1, 3

• Gabapentin can be used concurrently with IVIG as they work through different mechanisms 1
• Do not delay gabapentin initiation waiting for IVIG to "work first" 1
• Tricyclic antidepressants or carbamazepine are alternatives 1, 3

Autonomic Dysfunction

• Monitor for blood pressure/heart rate instability, pupillary dysfunction, bowel/bladder dysfunction 2, 1

Standard Preventive Measures

• Deep vein thrombosis prophylaxis 1
• Pressure ulcer prevention 1
• Treatment of constipation/ileus 1

Medications to AVOID

Avoid medications that worsen neuromuscular transmission: 2, 1

• β-blockers
• IV magnesium
• Fluoroquinolones
• Aminoglycosides
• Macrolides

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Managing Treatment Response

Expected Timeline

• Approximately 40% of patients do not improve in the first 4 weeks following treatment - this does not necessarily mean treatment failed, as progression might have been worse without therapy 1, 4, 6
• Recovery can continue for more than 3 years, with improvement possible even more than 5 years after onset 1, 4

Treatment-Related Fluctuations (TRFs)

• Occur in 6-10% of patients within 2 months after initial improvement 1, 4, 3
• Repeat full course of IVIg or plasma exchange for TRFs 1, 4, 3

When to Reconsider Diagnosis

Consider changing diagnosis to acute-onset CIDP (A-CIDP) if: 1, 3

• Progression continues after 8 weeks from onset
• Patient has three or more TRFs
• This occurs in approximately 5% of patients initially diagnosed with GBS 1, 3

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Special Populations

Children

• Use the same 5-day IVIg regimen (0.4 g/kg/day for 5 days) rather than accelerated 2-day protocols 4, 7
• IVIg is preferred over plasma exchange due to better tolerability and fewer complications 4, 7

Pregnant Women

• Both IVIg and plasma exchange are not contraindicated 4
• IVIg is generally preferred due to fewer monitoring requirements 4

Miller Fisher Syndrome

• Treatment is generally not recommended as most recover completely within 6 months without intervention 4
• Close monitoring is essential 4

Immune Checkpoint Inhibitor-Related GBS

• Permanently discontinue immune checkpoint inhibitor 2
• Consider concurrent corticosteroids (methylprednisolone 2-4 mg/kg/day) with IVIG or plasmapheresis 2
• Pulse corticosteroid dosing (methylprednisolone 1 g/day for 5 days) may also be considered for Grade 3-4 2

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Prognosis and Outcome Prediction

Expected Outcomes

• 80% of patients regain independent walking ability at 6 months 1, 4, 3
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 4, 3
• Advanced age and severe disease at onset are risk factors for poor outcome 1, 4

Prognostic Tools

• Use the modified Erasmus GBS outcome score (mEGOS) to predict probability of regaining walking ability 1, 3
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to calculate probability of requiring ventilation 4, 3

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Rehabilitation

Arrange a rehabilitation programme with a rehabilitation specialist, physiotherapist, and occupational therapist as a crucial step towards recovery. 1

• Exercise programmes including range-of-motion exercises, stationary cycling, walking, and strength training improve physical fitness, walking ability, and independence 1
• Screen for anxiety, depression, and hallucinations, which are frequent complications 1