What are the symptoms and treatment options for a patient with Guillain-Barré Syndrome (GBS)?

Guillain-Barré Syndrome: Clinical Presentation and Management

Clinical Symptoms

GBS presents with rapidly progressive bilateral ascending weakness starting in the legs and progressing to the arms and cranial muscles, accompanied by diminished or absent reflexes, with most patients reaching maximum disability within 2 weeks. 1

Cardinal Features

• Bilateral ascending weakness that typically begins in the lower extremities and ascends to involve upper limbs, trunk, and cranial muscles over days to 4 weeks (usually <2 weeks) 1, 2
• Areflexia or hyporeflexia affecting the lower limbs initially, then progressing to involve all extremities 1
• Distal paresthesias or sensory loss often precede or accompany the weakness, presenting as tingling, numbness, or sensory deficits 1
• Pain is present in approximately two-thirds of patients and can be muscular, radicular, or neuropathic in nature—this is often an early symptom that may precede weakness 3

Cranial Nerve and Autonomic Involvement

• Bilateral facial palsy is the most common cranial nerve manifestation, occurring due to the facial nerve's extensive myelin coverage and long intracranial course 3
• Dysautonomia manifests as blood pressure instability, cardiac arrhythmias, pupillary dysfunction, and bowel/bladder dysfunction 1
• Ophthalmoplegia and ataxia characterize Miller Fisher syndrome, a variant affecting 5-25% of cases 3

Respiratory Complications

• Respiratory failure develops in approximately 20% of patients and can occur rapidly without preceding dyspnea symptoms 1, 2
• Bulbar weakness with dysphagia and impaired cough increases aspiration risk 3

Atypical Presentations

• Young children (<6 years) may present with nonspecific features including irritability, refusal to bear weight, poorly localized pain, or unsteady gait 1
• Weakness can be asymmetrical or start simultaneously in all limbs rather than following the classic ascending pattern 1
• Isolated bilateral facial palsy can be the presenting feature before limb weakness develops 3

Treatment Options

Intravenous immunoglobulin (IVIg) 0.4 g/kg daily for 5 days or plasma exchange (200-250 ml/kg over 4-5 sessions) should be initiated in patients unable to walk unaided within 2-4 weeks of symptom onset—these treatments are equally effective. 1, 2

First-Line Immunotherapy

• IVIg dosing: 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) 1, 2
• Plasma exchange: 200-250 ml/kg divided over 4-5 sessions within 1-2 weeks 1, 4
• Treatment timing: Initiate within 2-4 weeks of symptom onset for patients unable to walk unaided 2, 4
• Do not delay treatment waiting for antibody test results or confirmatory studies if GBS is clinically suspected 3

Treatments NOT Recommended

• Corticosteroids alone are ineffective and should not be used 1, 4
• Sequential therapy with plasma exchange followed immediately by IVIg is not recommended 4
• Combination therapy does not provide additional benefit over monotherapy 4

Treatment-Related Fluctuations

• 6-10% of patients experience secondary deterioration within 2 months after initial improvement (treatment-related fluctuations) 1, 5
• Repeat treatment with a full course of IVIg or plasma exchange is common practice, though evidence for efficacy is limited 3, 4
• Approximately 40% of patients do not improve in the first 4 weeks, which does not necessarily indicate treatment failure 3

Adjunctive Management

• Pain management: Gabapentinoids, tricyclic antidepressants, or carbamazepine are weakly recommended for neuropathic pain 4
• Respiratory support: Mechanical ventilation is required in 20-25% of patients for days to months 1, 6
• ICU admission is indicated for patients with severe weakness, dysphagia, facial weakness, respiratory muscle weakness, or rapidly progressive symptoms 1

Critical Monitoring Parameters

• Respiratory function: Measure vital capacity, negative inspiratory force (NIF), and maximum inspiratory/expiratory pressures serially 3, 2
• "20/30/40 rule": Patient is at risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 3, 2
• Single breath count ≤19 predicts need for mechanical ventilation 3
• Cardiac monitoring: Continuous ECG and blood pressure monitoring for arrhythmias and autonomic instability 3
• Frequent neurochecks to assess progression of weakness and cranial nerve involvement 1, 5

Prognosis and Recovery

• 60-80% of patients regain independent walking ability at 6 months 1, 2
• Mortality remains 3-10% even with optimal care, primarily from cardiovascular and respiratory complications 1, 2
• Recovery continues for more than 3 years in many patients, with improvement possible even beyond 5 years 1, 3
• Recurrence is rare (2-5% of patients) but higher than the general population lifetime risk 3, 2
• Advanced age and severe disease at onset are risk factors for poor outcome 3

Clinical Variants and Their Prognosis

• Classic sensorimotor GBS (30-85% of cases): Full recovery expected in approximately 90% of cases, though recovery may take up to 2 years 3
• Pure motor variant (5-70% of cases): Motor weakness without sensory signs 3
• Miller Fisher syndrome (5-25% of cases): Characterized by ophthalmoplegia, ataxia, and areflexia 3

Critical Pitfalls to Avoid

• Do not dismiss GBS based on normal CSF protein in the first week, as albumino-cytological dissociation may not be present early in the disease course 3
• Bilateral simultaneous facial weakness is extremely rare in Bell's palsy and should immediately raise suspicion for GBS 3
• Marked persistent asymmetry, bladder dysfunction at onset, or marked CSF pleocytosis should prompt reconsideration of the diagnosis 3
• Consider A-CIDP (acute-onset chronic inflammatory demyelinating polyradiculoneuropathy) if progression continues beyond 8 weeks, which occurs in approximately 5% of patients initially diagnosed with GBS 4
• Recognize that patients with complete paralysis usually have intact consciousness, vision, and hearing—be mindful of bedside conversations and explain all procedures 3