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 unable to walk unaided, or with moderate to severe weakness, rapid progression, or any signs of respiratory compromise, dysphagia, facial weakness, or bulbar weakness. 1, 2

First-Line Immunotherapy

• IVIg is preferred over plasma exchange as first-line therapy because it is easier to administer, more widely available, has higher completion rates, and requires fewer monitoring considerations 1, 3

• Plasma exchange (200-250 ml/kg over 5 sessions, or 12-15 L in 4-5 exchanges over 1-2 weeks) remains equally effective and is an appropriate alternative, particularly when IVIg is unavailable or contraindicated 1, 4, 3

• Treatment must begin within 2 weeks of symptom onset for maximum effectiveness, though the European Academy of Neurology supports treatment up to 4 weeks after onset in patients unable to walk unaided 1, 3

• Do not use corticosteroids alone - they are ineffective and oral corticosteroids may worsen outcomes 1, 2, 3

• Do not combine plasma exchange followed immediately by IVIg - this provides no additional benefit 3

Critical Initial Assessment and Monitoring

Admission Requirements

• Admit all patients to a monitored inpatient unit with rapid transfer capability to intensive care, as approximately 25% of GBS patients develop respiratory failure requiring mechanical ventilation 1, 2, 4

Respiratory Monitoring Protocol

• Use the "20/30/40 rule" to assess respiratory failure risk: patient is at high risk if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1, 2, 4

• Calculate the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to predict probability of requiring mechanical ventilation within 1 week 1, 4

• Perform frequent vital capacity measurements, maximum inspiratory/expiratory pressures, and assess use of accessory respiratory muscles 1

Neurological Monitoring

• Perform serial neurological assessments using the Medical Research Council grading scale and GBS disability scale to monitor disease progression and treatment response 1, 4

• Monitor specifically for bulbar weakness (swallowing and coughing difficulties) to prevent aspiration 4

• Assess for autonomic dysfunction including heart rate variability, blood pressure instability, and bowel/bladder function 2, 4

Diagnostic Workup

• Obtain neurology consultation immediately to guide diagnostic approach and management 2

• Perform lumbar puncture with CSF analysis for cell count, differential, protein, glucose, and cultures - expect albuminocytologic dissociation (elevated protein with normal cell count) 2

• Conduct electrodiagnostic studies (nerve conduction studies and EMG) to evaluate polyneuropathy and distinguish AIDP from AMAN variants 2

• Perform MRI of spine with and without contrast to rule out compressive lesions and evaluate for nerve root enhancement/thickening 2

• Test for serum antiganglioside antibodies, particularly anti-GQ1b for suspected Miller Fisher syndrome 2, 3

• Screen for reversible neuropathy causes: HbA1c, vitamin B12, TSH, vitamin B6, folate, serum protein electrophoresis, and immunofixation 2

Medications to Avoid

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

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

IVIg Administration Details

Standard Dosing

• Administer 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) 1, 2

• Use ideal body weight for dosing calculations, not actual body weight, as IVIg distributes in plasma and extracellular fluid spaces that correlate with lean body mass rather than total body weight 1

• When total dose exceeds 80 grams, it may be administered over 3-5 days at 0.4 g/kg to improve tolerability 1

Pre-Treatment Precautions

• Check serum IgA levels before the first infusion - IgA deficiency increases anaphylaxis risk; if confirmed, use IVIg preparations with reduced IgA levels 1

• Monitor rigorously during and after each infusion for neurological function (motor strength, reflexes, bulbar symptoms) and potential adverse reactions 1

Supportive Care Essentials

Pain Management

• Manage neuropathic pain with gabapentin, pregabalin, or duloxetine 1, 2, 4, 3

• Avoid opioids for neuropathic pain management 1, 4

Preventive Measures

• Implement DVT prophylaxis in all immobilized patients 1, 4

• Provide pressure ulcer prevention with frequent repositioning 1, 4

• Address constipation/ileus, which is common in GBS patients 1, 4

• Evaluate for dysphagia and provide nutritional support if necessary 1

• Implement prevention strategies for hospital-acquired infections (pneumonia, UTIs) 2

• Provide corneal protection in patients with facial palsy to prevent corneal ulceration 2

• Prevent limb contractures with appropriate positioning and physical therapy 2

Managing Treatment Response and Complications

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

• About 80% of patients regain walking ability at 6 months after disease onset 1, 2

Treatment-Related Fluctuations (TRFs)

• TRFs occur in 6-10% of patients within 2 months of initial improvement 1, 2

• Repeat the full course of IVIg or plasma exchange for documented TRFs 1, 2

• Do not give a second IVIg course to patients with poor prognosis who have not had a TRF, as this does not improve outcomes 3

Distinguishing A-CIDP from GBS

• Consider changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) if progression continues after 8 weeks from onset, which occurs in approximately 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 shorter 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 additional precautions 1

Miller Fisher Syndrome

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

• Close monitoring remains essential 1

Immune Checkpoint Inhibitor-Related GBS

• Discontinue the causative agent permanently 1

• Consider concurrent corticosteroids (methylprednisolone 2-4 mg/kg/day) with IVIg or plasma exchange 1, 2

GBS with Suspected or Active Infection

• Do not delay initiation of IVIg or plasma exchange while attempting to rule out active infection - preceding infections have usually resolved before onset of weakness, and the autoimmune process typically begins 1-3 weeks after the triggering infection 1

• Start IVIg or plasma exchange immediately if GBS diagnosis is established 1

• Obtain appropriate cultures (blood, urine, respiratory) before starting antibiotics if clinically indicated 1

• Add targeted antimicrobials concurrently if active infection is documented or highly suspected based on clinical presentation 1

Prognosis and Mortality

• 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, mostly from cardiovascular and respiratory dysfunction, requiring continued vigilance even after apparent improvement 2, 4

• Use the modified Erasmus GBS outcome score (mEGOS) to assess individual patient prognosis for functional outcome 3