What is the recommended treatment and management for a patient suspected of having Guillain-Barré Syndrome?

Guillain-Barré Syndrome: Treatment and Management

Immediate Life-Threatening Assessment

Guillain-Barré syndrome is a medical emergency requiring immediate hospitalization with capability for rapid ICU transfer, as approximately 20% of patients develop respiratory failure that can occur rapidly and without obvious dyspnea. 1

Respiratory Monitoring

• Measure vital capacity, negative inspiratory force (NIF), and maximum inspiratory/expiratory pressures at presentation and serially 1, 2
• 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, 2
• Single breath count ≤19 predicts need for mechanical ventilation 2
• Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to calculate probability of requiring ventilation 1

Autonomic Monitoring

• Perform electrocardiography and continuously monitor heart rate and blood pressure for arrhythmias and blood pressure instability 2
• Monitor for pupillary dysfunction and bowel/bladder dysfunction 2

Neurological Assessment

• Grade muscle strength using Medical Research Council scale in neck, arms, and legs 2
• Assess functional disability using GBS disability scale 2
• Test swallowing and coughing ability to identify aspiration risk 2
• Assess for facial weakness (most commonly affected cranial nerve) and ophthalmoplegia 2
• Check corneal reflex in patients with facial palsy to prevent corneal ulceration 2

Diagnostic Workup

Essential Testing

• Cerebrospinal fluid analysis: Look for albumino-cytological dissociation (elevated protein with normal cell count), though this may be absent in the first week—do not dismiss GBS based on normal CSF protein early in disease course 1, 2
• Electrodiagnostic studies (nerve conduction studies and EMG): Look for sensorimotor polyradiculoneuropathy with reduced conduction velocities, reduced amplitudes, temporal dispersion, or conduction blocks 2
• "Sural sparing pattern" (normal sural sensory nerve action potential with abnormal median/ulnar responses) is typical for GBS 2
• MRI of spine with and without contrast to rule out compressive lesions and evaluate for nerve root enhancement/thickening 2

Laboratory Tests

• Complete blood count, glucose, electrolytes, kidney function, liver enzymes to exclude metabolic causes 2
• Serum creatine kinase (CK) - elevation suggests muscle involvement 2
• Screen for reversible neuropathy causes: HbA1c, vitamin B12, TSH, vitamin B6, folate 2
• Serum antiganglioside antibody tests for GBS subtypes (e.g., anti-GQ1b for Miller Fisher variant) 2, 3

Critical Diagnostic Pitfalls

• Do not wait for antibody test results before starting treatment if GBS is suspected 2
• Do not dismiss GBS based on normal CSF protein in the first week 1, 2
• Marked persistent asymmetry, bladder dysfunction at onset, or marked CSF pleocytosis should prompt reconsideration of the diagnosis 2
• Bilateral simultaneous facial weakness is extremely rare in Bell's palsy and should immediately raise suspicion for GBS 2

First-Line Immunotherapy

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

Treatment Options (Equally Effective)

Option 1: Intravenous Immunoglobulin (IVIg) - PREFERRED

• Dose: 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) 1, 3
• IVIg is preferred over plasma exchange because it is easier to administer, more widely available, and has higher completion rates 1
• In children, IVIg is preferred over plasma exchange due to better tolerability and fewer complications 1
• In pregnant women, IVIg is preferred due to fewer monitoring requirements 1

Option 2: Plasma Exchange

• Dose: 200-250 ml/kg over 4-5 sessions within 4 weeks of symptom onset 1, 3
• Equally effective as IVIg but more difficult to administer 1

IVIg Administration Details

• Use ideal body weight for dosing, not actual body weight in obese patients, as IVIG distributes in plasma and extracellular fluid spaces which correlate with lean body mass 1
• Check serum IgA levels before first infusion - IgA deficiency increases anaphylaxis risk; use preparations with reduced IgA levels if deficiency confirmed 1
• Monitor rigorously during and after each infusion for neurological function and adverse reactions 1
• When total dose exceeds 80 grams, may be administered over 3-5 days at 0.4 g/kg to improve tolerability 1

What NOT to Do

• Do NOT use corticosteroids alone - randomized controlled trials show no significant benefit and oral corticosteroids may have negative effects 1, 3
• Do NOT use plasma exchange followed immediately by IVIg - no additional benefit 3
• Do NOT give a second IVIg course routinely in patients with poor prognosis - not recommended 3

Treatment Response and Complications

Expected Timeline

• Approximately 40% of patients do not improve in the first 4 weeks following treatment - this does not necessarily mean treatment failed 1, 2, 4
• Disease progression typically reaches maximum disability within 2 weeks of symptom onset 2, 4
• Recovery can continue for more than 3 years, with improvement possible even more than 5 years after onset 2

Treatment-Related Fluctuations (TRFs)

• Occur in 6-10% of patients within 2 months after initial improvement 1, 4
• Defined as disease progression within 2 months following initial treatment-induced improvement or stabilization 2
• Repeating full course of IVIg or plasma exchange is common practice for TRFs 1, 4

When to Reconsider Diagnosis

• Consider changing diagnosis to acute-onset CIDP if progression continues after 8 weeks from onset or if patient has three or more TRFs - occurs in approximately 5% of patients initially diagnosed with GBS 2, 3

Supportive Care

Pain Management

• Use gabapentinoids (gabapentin, pregabalin) or duloxetine for neuropathic pain - preferred over opioids 1, 3
• Gabapentin can be used alongside IVIg without interaction - they work through different mechanisms 2
• Tricyclic antidepressants or carbamazepine are alternatives for neuropathic pain 2, 3
• Back and limb pain affects approximately two-thirds of patients and can be muscular, radicular, or neuropathic 2

Medications to AVOID

Avoid medications that worsen neuromuscular function: 1, 2

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

Standard Preventive Measures

• Deep vein thrombosis prophylaxis 1, 2
• Pressure ulcer prevention 1, 2
• Treatment of constipation/ileus 1, 2
• Evaluate dysphagia and provide nutritional support if necessary 1

Psychological Support

• Recognize that patients with GBS, even those with complete paralysis, usually have intact consciousness, vision, and hearing 2
• Screen for anxiety, depression, and hallucinations, which are frequent complications 2
• Be mindful of what is said at bedside and explain procedures to reduce anxiety 2

Rehabilitation

• Arrange rehabilitation programme with rehabilitation specialist, physiotherapist, and occupational therapist 2
• Exercise programmes including range-of-motion exercises, stationary cycling, walking, and strength training can improve physical fitness, walking ability, and independence 2

Prognosis

Outcomes

• 80% of patients regain independent walking ability at 6 months 1, 2
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 2, 4
• Approximately 25% require artificial ventilation during disease course 4
• About 20% are still unable to walk after 6 months 4
• Advanced age and severe disease at onset are risk factors for poor outcome 1, 2

Prognostic Tools

• Use the modified Erasmus GBS outcome score (mEGOS) to predict probability of regaining walking ability 2, 3
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess risk of requiring artificial ventilation 3

Recurrence

• Recurrence is rare (2-5% of patients) but higher than general population lifetime risk (0.1%) 2, 4
• Prior GBS is not a strict contraindication for vaccination 2

Special Considerations

Infection and Treatment

• Therapeutic plasma exchange or IVIg can be initiated even when infection is suspected - presence of active infection is not a contraindication 1
• Preceding infections have usually resolved before onset of weakness (typically 1-3 weeks after triggering infection) 1
• If active infection is documented, start appropriate antimicrobials concurrently with immunotherapy 1
• Do not withhold or delay immunotherapy while attempting to rule out active infection 1

Miller Fisher Syndrome

• Treatment is generally not recommended as most recover completely within 6 months without intervention, though close monitoring is essential 1
• Consider anti-GQ1b antibody testing when Miller Fisher syndrome is suspected (characterized by ophthalmoplegia, ataxia, and areflexia) 2, 3