What is the treatment for a patient with Guillain-Barré Syndrome (GBS) presenting with flaccid paralysis?

Treatment of Guillain-Barré Syndrome with Flaccid Paralysis

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg body weight daily for 5 consecutive days as first-line treatment for any patient with GBS presenting with flaccid paralysis who cannot walk unaided. 1, 2, 3

Immediate Priorities: Respiratory Assessment

Before or concurrent with immunotherapy initiation, respiratory function must be urgently assessed as respiratory failure can develop rapidly without obvious dyspnea 2, 3:

• Apply the "20/30/40 rule": Patient is at imminent risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 2, 3
• Perform single breath count: A count ≤19 predicts need for mechanical ventilation 2, 3
• Assess for use of accessory respiratory muscles 2
• Monitor continuously: Up to 22% of GBS patients require mechanical ventilation within the first week, and approximately 20% overall develop respiratory failure 1, 2

ICU Admission Criteria

Admit to intensive care unit if any of the following are present 2, 3:

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

First-Line Immunotherapy Selection

IVIg is preferred over plasma exchange because it is easier to administer, more widely available, has higher completion rates, and better tolerability with fewer complications 3, 4:

• IVIg dosing: 0.4 g/kg body weight daily for 5 consecutive days 1, 2, 3, 4
• Plasma exchange alternative: 200-250 ml plasma/kg body weight in 4-5 sessions over 1-2 weeks (total 12-15 L) if IVIg is contraindicated, not tolerated, or unavailable 1, 3, 4
• Both treatments are equally effective in reducing disability, but IVIg has practical advantages 1, 4
• Timing is critical: Treatment should be initiated as early as possible, ideally within 2 weeks of symptom onset 3, 4

What NOT to Do

Do not use corticosteroids alone or in combination as they have shown no significant benefit and may have negative effects on outcomes 1, 2, 3, 5, 4:

• Oral corticosteroids are not recommended 4
• IV corticosteroids are weakly recommended against 4
• Do not combine plasma exchange followed immediately by IVIg as this does not improve outcomes 4

Ongoing Monitoring During Hospitalization

Respiratory Function

• Continue serial vital capacity measurements 2
• Monitor for swallowing and coughing difficulties to prevent aspiration 2, 3, 5
• Arterial blood gas if respiratory compromise suspected 2

Neurological Assessment

• Assess muscle strength in neck, arms, and legs using Medical Research Council grading scale 2, 3
• Document functional disability using GBS disability scale 2, 3

Autonomic Dysfunction

• Continuous ECG monitoring for arrhythmias 3, 5
• Blood pressure monitoring for hypertension/hypotension 3, 5
• Monitor bowel and bladder function 3, 5
• Cardiovascular and respiratory dysfunction cause up to two-thirds of deaths in GBS 2, 5

Complications Prevention

• Deep vein thrombosis prophylaxis 2, 5
• Pressure ulcer prevention 2, 5
• Hospital-acquired infection surveillance 2, 5
• Pain management: Pain is common and significantly impacts quality of life; recognize and treat early 3, 5

Managing Treatment Failures and Fluctuations

Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 2, 3, 5:

• TRFs represent disease reactivation while the inflammatory phase continues 3
• For TRFs, repeat the full course of IVIg or switch to plasma exchange, though evidence supporting this is limited 3, 5
• About 40% of patients do not show improvement in the first 4 weeks, which doesn't necessarily indicate treatment ineffectiveness 5
• Consider changing diagnosis to acute-onset CIDP (A-CIDP) if repeated relapses occur or progression continues after 8 weeks from onset (occurs in approximately 5% of cases) 5, 4

Critical Medication Avoidance

Avoid medications that can worsen neuromuscular function 2:

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

Prognosis and Recovery

• 60-80% of patients regain independent walking ability at 6 months 1, 3
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 3
• Clinical improvement is usually most extensive in the first year but can continue for >5 years 1, 5
• Risk factors for poor outcome include advanced age and severe disease at onset 3

Early Rehabilitation

Initiate early multidisciplinary rehabilitation including physiotherapists, occupational therapists, speech therapists, and dietitians 3, 5:

• Include range-of-motion exercises, stationary cycling, walking, and strength training 3, 5
• Monitor exercise intensity closely: Overwork causes fatigue 3
• Pain management strategies include mobilization and drugs for neuropathic or nociceptive pain 5