What is the appropriate management and treatment for a patient suspected of having Guillain-Barré Syndrome (GBS)?

Management of Guillain-Barré Syndrome

Immediate Treatment Decision

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days immediately in any patient with GBS who cannot walk unaided or has moderate to severe weakness, especially with rapid progression, respiratory compromise, dysphagia, facial weakness, or bulbar weakness. 1, 2

Initial Assessment and Diagnostic Workup

Critical Clinical Evaluation

• Obtain neurology consultation immediately to guide diagnostic approach and management 2
• Assess for preceding infection history (diarrhea or respiratory infection within 1-3 weeks), as this increases likelihood of GBS 1, 3
• Perform lumbar puncture with CSF analysis for cell count, differential, protein, glucose, and cultures—expect elevated protein with normal cell count (albuminocytologic dissociation) 2
• Order electrodiagnostic studies (nerve conduction studies and EMG) to evaluate polyneuropathy pattern 2
• Obtain MRI of spine with and without contrast to rule out compressive lesions and evaluate for nerve root enhancement/thickening 2

Laboratory Testing

• Test serum antiganglioside antibodies for GBS subtypes (anti-GQ1b for Miller Fisher variant) 2
• Screen for reversible neuropathy causes: HbA1c, vitamin B12, TSH, vitamin B6, folate, serum protein electrophoresis, and immunofixation 2
• Verify serum IgA levels before first IVIg infusion, as IgA deficiency increases anaphylaxis risk; use IVIg preparations with reduced IgA levels if deficiency confirmed 1

First-Line Immunotherapy

IVIg Protocol (Preferred)

• Administer IVIg 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) as first-line treatment 1, 2
• IVIg is preferred over plasma exchange due to easier administration, wider availability, higher completion rates, and better tolerability 1, 3
• Use ideal body weight for dosing in obese patients, as IVIg distributes in plasma and extracellular fluid spaces correlating with lean body mass, not adipose tissue 1
• When total dose exceeds 80 grams, may administer over 3-5 days at 0.4 g/kg to improve tolerability 1

Plasma Exchange Alternative

• Plasma exchange (200-250 mL/kg over 5 sessions in 1-2 weeks) is equally effective and appropriate as alternative, particularly in resource-limited settings where cost is a major factor 1, 2, 4
• Do not combine plasma exchange followed immediately by IVIg, as this provides no additional benefit 3

Treatment Timing

• Initiate treatment within 2 weeks of symptom onset for maximum effectiveness 4, 3
• Treatment should not be delayed even if active infection is suspected, as preceding infections have usually resolved before weakness onset 1
• If active infection is documented, start appropriate antimicrobials concurrently with immunotherapy without delaying IVIg or plasma exchange 1

What NOT to Use

• Do not use corticosteroids alone—they are ineffective and oral corticosteroids may worsen outcomes 1, 3
• IV corticosteroids are also not recommended for idiopathic GBS (may be considered only for immune checkpoint inhibitor-related GBS with methylprednisolone 2-4 mg/kg/day) 2
• Do not administer a second IVIg course in patients with poor prognosis, as this does not improve outcomes 3

Hospital Admission and Monitoring

Admission Criteria

• Admit all GBS patients to an inpatient unit with rapid transfer capability to intensive care, as approximately 25% develop respiratory failure 1, 4, 3
• Even patients receiving treatment can develop respiratory compromise during therapy 1

Respiratory Monitoring Protocol

• Use the "20/30/40 rule" to assess respiratory failure risk: patient is at risk if vital capacity <20 mL/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1, 2, 4
• Calculate Erasmus GBS Respiratory Insufficiency Score (EGRIS) to predict probability of requiring mechanical ventilation within 1 week 1, 4
• Perform frequent pulmonary function monitoring including vital capacity, maximum inspiratory/expiratory pressures, and assessment of accessory respiratory muscle use 1, 2

Neurological Monitoring

• Perform serial neurologic examinations using Medical Research Council grading scale and GBS disability scale 4
• Monitor rigorously during and after each IVIg infusion for neurological function, including motor strength, reflexes, and bulbar symptoms 1
• Monitor for autonomic dysfunction: heart rate, blood pressure, bowel and bladder function 2

Bulbar and Cranial Nerve Assessment

• Monitor swallowing and coughing difficulties to prevent aspiration 4
• Evaluate for dysphagia and provide nutritional support if necessary 1
• Assess for corneal ulceration in patients with facial palsy 2

Medications to Avoid

Avoid the following medications that worsen neuromuscular function: 1, 2, 4

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

Supportive Care and Complication Prevention

Pain Management

• Manage neuropathic pain with gabapentin, pregabalin, or duloxetine—avoid opioids 1, 2, 4
• These gabapentinoids, tricyclic antidepressants, or carbamazepine are weakly recommended for pain treatment 3

Preventive Measures

• Implement DVT prophylaxis for all immobilized patients 1, 4
• Provide pressure ulcer prevention with frequent repositioning 2, 4
• Address constipation/ileus, which is common in GBS patients 1, 4
• Implement preventive measures for hospital-acquired infections 2
• Prevent limb contractures in paralyzed patients 2

Managing Treatment Response and Fluctuations

Expected Timeline

• Approximately 40% of patients do not improve in the first 4 weeks following treatment—this does not necessarily indicate treatment failure 1, 2, 4
• Treatment should begin showing effects within 2-4 weeks in most responders 1

Treatment-Related Fluctuations

• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1, 2, 4
• Repeat the full course of IVIg or plasma exchange for TRFs 1, 2, 4

Consideration of A-CIDP

• Consider changing diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS 3

Special Populations

Pediatric Patients

• Use the same 5-day IVIg regimen (0.4 g/kg/day for 5 days) in children rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with 2-day regimens 1
• IVIg is preferred over plasma exchange in children due to better tolerability and fewer complications 1

Pregnant Women

• Both IVIg and plasma exchange are not contraindicated in pregnancy 1
• IVIg is generally preferred due to fewer monitoring requirements and considerations 1

Miller Fisher Syndrome

• Treatment is generally not recommended for Miller Fisher Syndrome, as most patients recover completely within 6 months without intervention, though close monitoring is essential 1

AMSAN Variant

• Treat AMSAN (acute motor sensory axonal neuropathy) with the same IVIg protocol as standard GBS 4
• AMSAN patients often present later and more severely than AIDP patients, making early recognition crucial 4
• Up to two-thirds of deaths in AMSAN occur during the recovery phase from cardiovascular and respiratory dysfunction, requiring continued vigilance even after apparent improvement 4

Prognosis

• Approximately 80% of patients regain walking ability at 6 months after disease onset 1, 2, 4
• Mortality occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications 1, 2, 4
• Risk factors for mortality include advanced age and severe disease at onset 1
• Up to two-thirds of deaths occur during the recovery phase, necessitating continued vigilance even after improvement 2
• Use the modified Erasmus GBS outcome score (mEGOS) to assess individual patient prognosis 3