What is the recommended treatment for a patient with suspected infection-related Guillain-Barré Syndrome (GBS)?

Treatment of Guillain-Barré Syndrome Following Infection

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg daily for 5 consecutive days as soon as the patient cannot walk unaided, ideally within 2 weeks of symptom onset. 1, 2, 3

First-Line Immunotherapy

IVIg is the preferred first-line treatment over plasma exchange because it is easier to administer, more widely available, has higher completion rates, better tolerability, and fewer complications—particularly important in children and pregnant women. 4, 1, 2, 3

• Plasma exchange (200-250 ml/kg over 4-5 sessions) is equally effective but reserved as an alternative when IVIg is contraindicated or unavailable. 4, 2, 3
• Do not use corticosteroids alone—eight randomized controlled trials showed no significant benefit, and oral corticosteroids may worsen outcomes. 4, 1, 3
• Do not combine plasma exchange followed immediately by IVIg—this is no more effective than either treatment alone. 4, 3

Treatment Timing

• Start treatment as early as possible within 2 weeks of symptom onset for patients unable to walk unaided. 1, 3
• Treatment can still be considered 2-4 weeks after onset, though evidence is stronger for earlier intervention. 3

Critical Respiratory Monitoring and ICU Admission

Apply the "20/30/40 rule" immediately and serially to identify imminent respiratory failure risk: 1, 2

• Vital capacity <20 ml/kg
• Maximum inspiratory pressure <30 cmH₂O
• Maximum expiratory pressure <40 cmH₂O

Single breath count ≤19 predicts need for mechanical ventilation. 1, 2

ICU Admission Criteria

Admit to ICU if any of the following are present: 1, 2

• Evolving respiratory distress with imminent respiratory insufficiency
• Severe autonomic cardiovascular dysfunction
• Severe swallowing dysfunction or diminished cough reflex
• Rapid progression of weakness

Up to 30% of patients develop respiratory failure requiring mechanical ventilation, which can occur rapidly and sometimes without obvious dyspnea. 1, 2, 5

Autonomic and Cardiovascular Monitoring

Continuous ECG monitoring for arrhythmias and blood pressure monitoring for hypertension/hypotension is essential. 1, 2

• Cardiac arrhythmias and blood pressure instability from autonomic dysfunction can be life-threatening. 4, 2
• Monitor bowel and bladder function, as autonomic dysregulation affects gastrointestinal motility. 6

Medications to Avoid

Avoid medications that worsen neuromuscular function: 1

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

Managing Treatment-Related Fluctuations

Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement, representing disease reactivation while the inflammatory phase continues. 4, 1, 2

• For TRFs, repeat the full course of IVIg or switch to plasma exchange, though evidence supporting this practice is limited. 4, 1, 2
• About 40% of patients do not improve in the first 4 weeks following treatment—this does not necessarily indicate treatment ineffectiveness. 1

A second IVIg course is not recommended for GBS patients with a poor prognosis based on current evidence. 3

Special Populations

Children

• Use the same 5-day IVIg regimen (0.4 g/kg/day for 5 days) rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens. 4, 1
• IVIg is preferred over plasma exchange due to fewer monitoring requirements and lower complication rates. 4

Pregnant Women

• IVIg is preferred over plasma exchange due to fewer monitoring requirements, though neither is contraindicated. 4, 1

Immune Checkpoint Inhibitor-Related GBS

• Discontinue the causative agent permanently. 1
• Consider concurrent corticosteroids with IVIg or plasma exchange—unlike canonical GBS, ICI-mediated GBS has been successfully managed using corticosteroid therapy. 4

Pain Management

Severe pain occurs in at least one-third of patients 1 year after onset and can persist >10 years, characterized by muscle pain in lower back and limbs, painful paresthesias, arthralgia, and radicular pain. 4, 1

For neuropathic pain, use: 1, 3

• Gabapentinoids (gabapentin, pregabalin)
• Tricyclic antidepressants
• Carbamazepine

Encourage early mobilization for muscle pain and arthralgia related to immobility. 1

Rehabilitation and Long-Term Management

Initiate early rehabilitation with a multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians. 1

Exercise programs should include: 4, 1

• Range-of-motion exercises
• Stationary cycling
• Walking and strength training

Monitor exercise intensity closely—overwork causes fatigue. 4, 1

• Fatigue affects 60-80% of patients and is often one of the most disabling complaints. 4, 1
• Graded, supervised exercise programs help reduce fatigue. 4, 1

Prognosis

About 80% of patients regain independent walking ability at 6 months. 4, 1, 2

Mortality occurs in 3-10% of cases, most commonly from cardiovascular and respiratory complications in both acute and recovery phases. 4, 1, 2

Risk factors for mortality and poor outcome: 4, 1, 2

• Advanced age
• Severe disease at onset

Use the modified Erasmus GBS outcome score (mEGOS) to calculate probability of regaining walking ability in individual patients. 4, 1, 3

Recovery may continue for >5 years after disease onset for residual complaints. 4, 1

Recurrent GBS is rare (2-5% of patients), but this is still higher than the lifetime risk in the general population (0.1%). 4, 1

Management of Infection-Related Complications

Antimicrobial or antiviral treatment can be considered in patients with GBS who have an ongoing infection, though preceding infections have usually resolved before the onset of weakness. 4

• For gastroparesis related to autonomic dysfunction: use dietary modifications (frequent smaller meals, low fat and fiber), prokinetic agents (metoclopramide, erythromycin), and antiemetics (phenothiazines, 5-HT3 receptor antagonists). 6
• Gastroparesis symptoms will likely improve as the underlying GBS is treated with IVIg or plasma exchange. 6