What is the recommended first‑line treatment and overall management for an adult with Guillain‑Barré syndrome without contraindications?

Guillain-Barré Syndrome: First-Line Treatment and Management

First-Line Immunotherapy

Administer intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) for any adult patient unable to walk independently within 2–4 weeks of symptom onset. 1, 2

• IVIg is preferred over plasma exchange because it is easier to administer, more widely available, has higher completion rates, and requires fewer monitoring considerations. 1
• Plasma exchange (200–250 mL/kg over 4–5 sessions) is equally effective but reserved for situations where IVIg is contraindicated or unavailable. 3, 1
• Treatment should be initiated as early as possible in the disease course to maximize effectiveness, ideally within 2 weeks of symptom onset. 1, 4
• Do not use corticosteroids alone for idiopathic GBS—randomized controlled trials show no significant benefit and oral corticosteroids may worsen outcomes. 1, 2

Immediate Assessment Priorities

Evaluate respiratory function and autonomic stability immediately upon presentation, as these determine mortality risk and need for ICU-level care. 1, 5

Respiratory Monitoring

• Measure vital capacity, negative inspiratory force (NIF), and maximum inspiratory/expiratory pressures at presentation and serially. 1
• Apply the "20/30/40 rule": patient is at high risk of respiratory failure if vital capacity <20 mL/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O. 1
• Single breath count ≤19 predicts need for mechanical ventilation. 1
• Approximately 20% of patients develop respiratory failure requiring mechanical ventilation, which can occur rapidly without obvious dyspnea. 5

Autonomic Monitoring

• Perform continuous electrocardiography and blood pressure monitoring for arrhythmias and hemodynamic instability. 1, 5
• Monitor for pupillary dysfunction, bowel/bladder dysfunction, and other signs of dysautonomia. 3, 1

Admission Criteria

Admit all patients with Grade 3–4 disease to an inpatient unit with rapid ICU transfer capability: severe weakness limiting self-care, dysphagia, facial weakness, respiratory muscle weakness, or rapidly progressive symptoms. 1, 5

• Even patients with moderate symptoms (Grade 2) require neurology consultation and close inpatient monitoring. 1, 5

Diagnostic Workup

Obtain immediate neurology consultation for every suspected GBS case. 5

Essential Testing

• 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 exclude GBS based on normal CSF protein early in disease. 1, 5, 2
• Electrodiagnostic studies (nerve conduction studies and EMG) to support diagnosis and identify the "sural sparing pattern" (normal sural sensory nerve action potential with abnormal median/ulnar responses). 1, 5, 2
• Do not delay treatment while awaiting antiganglioside antibody test results if clinical suspicion is high. 5, 2

Exclude Alternative Diagnoses

• Order complete blood count, glucose, electrolytes, renal function, liver enzymes, HbA1c, vitamin B12, TSH, vitamin B6, and folate to rule out metabolic causes. 1, 5
• Consider MRI of spine with contrast to exclude compressive lesions and assess for nerve root enhancement. 5, 2

Medications to Avoid

Discontinue or avoid medications that worsen neuromuscular function: β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides. 1, 5, 4

Pain Management

Initiate gabapentinoids (gabapentin or pregabalin) or duloxetine immediately for neuropathic pain—these can be safely co-administered with IVIg without drug interactions. 1, 2

• Pain affects two-thirds of patients and can be muscular, radicular, or neuropathic. 3, 5
• Avoid opioids as first-line agents. 1

Treatment Response and Fluctuations

Approximately 40% of patients do not improve in the first 4 weeks following treatment—this does not necessarily indicate treatment failure, as progression might have been worse without therapy. 1, 5, 4

Treatment-Related Fluctuations (TRFs)

• TRFs occur in 6–10% of patients within 2 months after initial improvement or stabilization. 1, 5, 4
• Repeat a full course of IVIg or plasma exchange for TRFs, though high-quality evidence supporting this approach is limited. 1, 5
• 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. 5, 2

Supportive Care

Daily Management

• Perform daily neurological examinations to track motor strength, reflexes, and bulbar symptoms. 1, 5
• Provide DVT prophylaxis, pressure ulcer prevention, and monitor for hospital-acquired infections. 1, 5
• Assess swallowing function and provide nutritional support if dysphagia is present. 1, 5
• Treat constipation/ileus proactively. 1, 5

Psychological Support

• Recognize that patients with GBS, even those with complete paralysis, usually have intact consciousness, vision, and hearing. 1
• Screen for anxiety, depression, and hallucinations, which are frequent complications. 1

Prognosis

Approximately 80% of patients regain independent walking ability at 6 months, though recovery can continue for more than 3 years and improvements are possible even beyond 5 years. 3, 1, 5

• Mortality is 3–10%, primarily from cardiovascular and respiratory complications. 3, 1, 5
• Advanced age and severe disease at onset are risk factors for poor outcome. 1, 5
• Use the modified Erasmus GBS outcome score (mEGOS) on admission to predict probability of regaining walking ability. 3, 5
• Recurrence is rare (2–5% of patients) but higher than the general population lifetime risk (0.1%). 3, 1

Rehabilitation

Arrange a structured rehabilitation program with physiotherapists, occupational therapists, and rehabilitation specialists as soon as the patient is medically stable. 5

• Exercise programs (range-of-motion, stationary cycling, walking, strength training) improve physical fitness, walking ability, and independence in activities of daily living. 5
• Fatigue affects 60–80% of survivors and is a major disabling symptom requiring long-term management. 5
• Severe pain affects at least one-third of patients at 1 year and may persist for more than a decade. 5

Special Populations

Immune Checkpoint Inhibitor-Related GBS

• Permanently discontinue the immune checkpoint inhibitor when GBS is diagnosed. 1, 5
• Add concurrent corticosteroids (methylprednisolone 2–4 mg/kg/day) to IVIg or plasma exchange. 1, 5
• For Grade 3–4 severity, consider pulse corticosteroid therapy (methylprednisolone 1 g/day for 5 days) as an adjunct. 1, 5

Pregnant Women

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

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. 1
• IVIg is preferred over plasma exchange in children due to better tolerability and fewer complications. 1

Obese Patients

• Use ideal body weight for IVIg dosing, as adipose tissue does not significantly contribute to the volume of distribution for immunoglobulin. 1

Common Pitfalls

• Do not dismiss GBS based on normal CSF protein in the first week—albumino-cytological dissociation may not appear until the end of the first week or later. 1, 5, 2
• Do not wait for electrodiagnostic confirmation if clinical suspicion is high—nerve conduction studies may be normal early in the disease course. 1, 5
• Marked persistent asymmetry, bladder dysfunction at onset, or marked CSF pleocytosis should prompt reconsideration of the diagnosis. 3, 5
• Young children (<6 years) can present atypically with poorly localized pain, refusal to bear weight, irritability, or unsteady gait—failure to recognize these signs causes diagnostic delay. 3