What is the management and treatment approach for a patient diagnosed with Guillain-Barré Syndrome (GBS)?

Management and Treatment of Guillain-Barré Syndrome

For patients with Guillain-Barré Syndrome who cannot walk unaided within 2-4 weeks of symptom onset, initiate either intravenous immunoglobulin (IVIg) 0.4 g/kg/day for 5 consecutive days (total 2 g/kg) or plasma exchange (200-250 mL/kg over 4-5 sessions), as both are equally effective first-line treatments. 1, 2

Immediate Assessment and Stabilization

Critical Life-Threatening Evaluation

• Assess respiratory function immediately using vital capacity, negative inspiratory force (NIF), and maximum inspiratory/expiratory pressures at presentation and serially 3
• Apply the "20/30/40 rule": patient is at high risk for respiratory failure if vital capacity <20 mL/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 3
• Single breath count ≤19 predicts need for mechanical ventilation 3
• Approximately 20% of patients develop respiratory failure requiring mechanical ventilation, which can occur rapidly and sometimes without obvious dyspnea 1, 4, 5
• Perform continuous cardiac monitoring and ECG to detect arrhythmias and blood pressure instability from autonomic dysfunction 1, 3

Hospital Admission Criteria

• All grades of GBS warrant workup and intervention given potential for progressive disease leading to respiratory compromise 1
• Admit to inpatient unit with capability for rapid transfer to ICU-level monitoring for Grade 3-4 disease (severe weakness limiting self-care, any dysphagia, facial weakness, respiratory muscle weakness, or rapidly progressive symptoms) 1
• Even patients with moderate symptoms (Grade 2) require neurology consultation and close monitoring 1

Diagnostic Workup

Essential Investigations

• Neurology consultation should be obtained for all suspected GBS cases 1, 3
• 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 the disease course 1, 3
• CSF should also be analyzed for cell count and differential, cytology for malignant cells, glucose, and viral/bacterial cultures 1
• Electrodiagnostic studies (nerve conduction studies and EMG) to evaluate polyneuropathy and classify the subtype (AIDP, AMAN, or AMSAN) 1, 4
• Look for "sural sparing pattern" (normal sural sensory nerve action potential with abnormal median/ulnar responses), which is typical for GBS 3
• MRI of spine with and without contrast to rule out compressive lesions and evaluate for nerve root enhancement/thickening 1
• Serum antiganglioside antibody tests for GBS subtypes (e.g., anti-GQ1b for Miller Fisher variant associated with ataxia and ophthalmoplegia) 1
• Pulmonary function testing (NIF or vital capacity) 1

Laboratory Screening

• Complete blood count, glucose, electrolytes, kidney function, liver enzymes to exclude metabolic causes 3
• Screen for reversible neuropathy causes: HbA1c, vitamin B12, TSH, vitamin B6, folate 1
• Serum creatine kinase (CK) to assess for muscle involvement 3

First-Line Immunotherapy

Treatment Indications and Timing

• Initiate treatment in patients unable to walk unaided within 2-4 weeks of symptom onset 1, 5, 2
• Do not wait for antibody test results before starting treatment if GBS is suspected 3

IVIg Protocol (Preferred for Practical Reasons)

• IVIg 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) 1, 2
• IVIg is generally preferred over plasma exchange for practical reasons, though both are equally effective 2, 6

Plasma Exchange Protocol

• Plasma exchange 200-250 mL/kg over 4-5 sessions within 4 weeks of symptom onset 1, 2
• Note: Plasma exchange immediately after IVIg will remove immunoglobulin, so avoid this sequence 1

Corticosteroid Considerations

• Corticosteroids are NOT recommended for idiopathic GBS 1, 2, 7
• However, in immune checkpoint inhibitor-related GBS, a trial of corticosteroids is reasonable (methylprednisolone 2-4 mg/kg/day) 1
• For severe Grade 3-4 immune checkpoint inhibitor-related GBS, pulse steroid dosing (methylprednisolone 1 g daily for 5 days) may be considered along with IVIg or plasmapheresis 1

What NOT to Do

• Do NOT give a second course of IVIg routinely in GBS patients with poor prognosis—evidence does not support this approach 2
• Do NOT use PE followed immediately by IVIg—this combination is not recommended 2
• Do NOT use oral or IV corticosteroids alone—they are ineffective in GBS 2, 7

Managing Treatment Response and Complications

Expected Response

• Approximately 40% of patients do not improve in the first 4 weeks following treatment—this does not necessarily mean treatment failed, as progression might have been worse without therapy 1
• Treatment approach does not differ based on electrophysiological subtype (AIDP, AMAN, or AMSAN)—both IVIg and plasma exchange are first-line regardless 4

Treatment-Related Fluctuations (TRFs)

• TRFs occur in 6-10% of patients, defined as disease progression within 2 months following initial treatment-induced improvement or stabilization 1
• Repeating the full course of IVIg or plasma exchange is common practice for TRFs, though evidence is lacking 1, 3

Acute-Onset CIDP

• Consider changing diagnosis to acute-onset CIDP if progression continues after 8 weeks from onset or if patient has three or more TRFs—this occurs in approximately 5% of patients initially diagnosed with GBS 1, 2

Supportive Care and Complication Management

Respiratory Management

• Frequent pulmonary function assessment with serial vital capacity and NIF measurements 1
• Be prepared for rapid intubation—respiratory failure can occur without preceding dyspnea 4

Autonomic Dysfunction

• Monitor for concurrent autonomic dysfunction including blood pressure/heart rate instability, pupillary dysfunction, bowel/bladder dysfunction 1
• Continuous cardiac monitoring is critical, especially during the acute and recovery phases 1

Pain Management

• Use gabapentinoids (gabapentin, pregabalin) or duloxetine for neuropathic pain—these are weakly recommended 1, 2
• Tricyclic antidepressants or carbamazepine may also be considered 2
• Nonopioid management is preferred 1
• Pain affects approximately two-thirds of patients and can be muscular, radicular, or neuropathic 3

Other Supportive Measures

• Daily neurologic evaluation 1
• Treatment of constipation/ileus 1
• Assess swallowing and coughing ability to identify aspiration risk 3
• Check corneal reflex in patients with facial palsy to prevent corneal ulceration 3
• Standard preventive measures for pressure ulcers, hospital-acquired infections, and deep vein thrombosis 1

Psychological Support

• Screen for anxiety, depression, and hallucinations, which are frequent complications 1, 3
• Recognize that patients with GBS, even those with complete paralysis, usually have intact consciousness, vision, and hearing 1, 3
• Be mindful of what is said at bedside and explain procedures to reduce anxiety 1, 3

Medications to Avoid

• Avoid medications that can worsen neuromuscular transmission: β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides 1

Prognosis and Outcome Prediction

Expected Recovery

• 80% of patients regain independent walking ability at 6 months 1, 3
• Recovery can continue for more than 3 years, with improvement possible even more than 5 years after onset 1, 3
• Full recovery expected in approximately 90% of cases 3

Mortality and Risk Factors

• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 4, 3
• Advanced age and severe disease at onset are risk factors for poor outcome 1, 3

Prognostic Tools

• Use the modified Erasmus GBS outcome score (mEGOS) to predict probability of regaining walking ability 1, 2
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess risk of requiring artificial ventilation 2

Recurrence

• Recurrence is rare, occurring in only 2-5% of cases 3, 6

Common Pitfalls to Avoid

• Do not dismiss GBS based on normal CSF protein in the first week—albumino-cytological dissociation may not be present early 3
• Do not delay treatment waiting for confirmatory tests—start immunotherapy based on clinical suspicion 3
• Do not assume treatment failure if no improvement in first 4 weeks—this is common and does not indicate ineffective therapy 1
• Marked persistent asymmetry, bladder dysfunction at onset, or marked CSF pleocytosis should prompt reconsideration of the diagnosis 3
• Nadir reached in less than 24 hours should cast doubt on the diagnosis of GBS—this is atypically rapid 5