What is the treatment for Guillain-Barré Syndrome (GBS)?

Guillain-Barré Syndrome: Treatment and Management

Acute Immunotherapy

Treat all patients unable to walk unaided with either intravenous immunoglobulin (IVIg) 0.4 g/kg/day for 5 consecutive days (total 2 g/kg) OR plasma exchange (12-15 L over 4-5 sessions) within 2 weeks of symptom onset. 1

• Both IVIg and plasma exchange are equally effective first-line treatments and the choice between them is primarily based on practical considerations and local availability 1, 2
• IVIg is typically preferred in clinical practice due to easier administration, better availability, and fewer complications compared to plasma exchange 3
• Treatment initiated within the first 2 weeks is associated with better outcomes, though benefit may extend to 4 weeks for plasma exchange 4, 1
• Do NOT combine plasma exchange followed immediately by IVIg as this approach does not improve outcomes 1
• Do NOT use corticosteroids alone as they are ineffective in GBS and may worsen outcomes 1, 3

Treatment Does Not Vary by Subtype

• The electrophysiological subtype (AIDP vs AMAN vs AMSAN) does not influence treatment selection—both IVIg and plasma exchange are first-line regardless of demyelinating versus axonal features 5, 2
• Approximately one-third of patients cannot be classified electrophysiologically at initial presentation, reinforcing that treatment decisions should not wait for subtype determination 5

Critical Monitoring and Supportive Care

Respiratory Monitoring

• Approximately 20% of patients develop respiratory failure requiring mechanical ventilation, which can occur rapidly and sometimes without obvious dyspnea 5, 4
• Monitor vital capacity and negative inspiratory force regularly to assess respiratory function 5
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to predict risk of requiring artificial ventilation 1
• Be prepared for emergent intubation as respiratory decline can be precipitous 6

Autonomic Monitoring

• Continuous cardiac monitoring is critical as cardiac arrhythmias and blood pressure instability from autonomic dysfunction can be life-threatening 5, 4
• Dysautonomia includes blood pressure/heart rate fluctuations, pupillary dysfunction, and bowel/bladder dysfunction 5
• Significant hemodynamic instability may require ICU-level care even in patients without respiratory failure 6

Prevention of Complications

• Implement standard deep vein thrombosis prophylaxis for all bed-bound patients 7
• Monitor for dysphagia in patients with bulbar palsy to prevent aspiration 7
• Protect corneas in patients with facial palsy to prevent ulceration 7
• Prevent limb contractures, ossification, and pressure palsies through early mobilization and positioning 7

Management of Treatment-Related Fluctuations

• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months following initial treatment-induced improvement 7, 4
• TRFs indicate the treatment effect has worn off while inflammation continues, and repeating the full course of IVIg or plasma exchange is common practice 7
• Distinguish TRFs from insufficient initial response (40% of patients do not improve in first 4 weeks) or progression to acute-onset CIDP 7
• Consider diagnosis of acute-onset CIDP if progression continues beyond 8 weeks or if three or more TRFs occur (affects ~5% of GBS patients) 7, 1

Second Course IVIg

• Do NOT routinely give a second course of IVIg to patients with poor prognosis as current evidence does not support this approach 1
• Clinical trials investigating second-dose IVIg are ongoing but results are not yet available 7

Pain Management

• Severe pain occurs in at least one-third of patients and includes muscle pain, radicular pain, painful paresthesias, and arthralgia 7
• Pain can precede weakness and may confuse the diagnosis 8
• Use gabapentinoids, tricyclic antidepressants, or carbamazepine for neuropathic pain 1
• Encourage early mobilization for muscle pain and arthralgia related to immobility 7
• Recognize and treat pain early as it significantly impacts wellbeing, especially in ICU patients with limited communication 7

Psychological Support

• Early recognition and treatment of anxiety, depression, and hallucinations is crucial as these symptoms are frequent and significantly impact recovery 7
• Patients with complete paralysis usually have intact consciousness, vision, and hearing—be mindful of bedside conversations and explain all procedures to reduce anxiety 7
• Referral to psychology or psychiatry may benefit some patients, as mental status influences physical recovery 7
• Provide accurate prognostic information: 80% regain independent walking at 6 months, and recurrence risk is only 2-5% 7

Rehabilitation and Long-Term Management

Structured Rehabilitation Program

• Arrange multidisciplinary rehabilitation with physiotherapy, occupational therapy, and speech therapy before discharge 7
• Exercise programs including range-of-motion exercises, stationary cycling, walking, and strength training improve physical fitness, walking ability, and independence 7
• Monitor exercise intensity closely as overwork can worsen fatigue 7

Fatigue Management

• Fatigue affects 60-80% of patients and is often the most disabling long-term complaint, unrelated to residual motor deficits 7
• Rule out other causes before attributing fatigue to GBS 7
• Graded, supervised exercise programs reduce fatigue 7
• No specific pharmacological treatment for fatigue is recommended 1

Prognosis

• Use the modified Erasmus GBS Outcome Score (mEGOS) to predict individual patient outcomes including probability of regaining independent walking 7, 1
• Most patients show extensive recovery, especially in the first year, even those who were tetraplegic or ventilator-dependent 7
• Mortality remains 3-10% despite optimal care, primarily from cardiovascular and respiratory complications in both acute and recovery phases 7, 5
• Risk factors for mortality include advanced age and severe disease at onset 7
• Recovery can continue beyond 5 years for neuropathic pain, weakness, and fatigue 7

Vaccination Considerations

• Prior GBS is not an absolute contraindication to vaccination 7
• Consult experts for patients diagnosed with GBS <1 year before planned vaccination or who developed GBS shortly after the same vaccine previously 7
• Weigh benefits of vaccination (e.g., influenza in elderly) against small theoretical recurrence risk 7
• Recurrence affects 2-5% of patients, higher than the 0.1% lifetime risk in the general population 7