What is the management, diagnostic approach, goal of management, prognosis, and complications of Guillain-Barré Syndrome (GBS)?

Management of Guillain-Barré Syndrome (GBS)

The management of Guillain-Barré Syndrome requires early immunotherapy with either intravenous immunoglobulin (IVIg) or plasma exchange, along with comprehensive supportive care and monitoring for complications, as these interventions significantly reduce morbidity and mortality.

Diagnostic Approach

Clinical Features

• Progressive bilateral weakness of legs and/or arms with decreased or absent reflexes, typically reaching maximum disability within 2 weeks 1
• Distal paresthesias or sensory loss often precede or accompany weakness 1
• Dysautonomia is common (blood pressure/heart rate instability, pupillary dysfunction, bowel/bladder dysfunction) 1
• Pain (muscular, radicular, or neuropathic) is frequently reported 1

Diagnostic Workup

• Lumbar puncture: Typically shows elevated protein with normal white blood cell count (albuminocytologic dissociation) 1
• Electrodiagnostic testing: Nerve conduction studies to support diagnosis and differentiate subtypes (AIDP, AMAN, AMSAN) 1, 2
• Consider anti-ganglioside antibody testing (particularly anti-GQ1b for Miller Fisher variant) 2
• MRI or ultrasound imaging should be considered in atypical cases 2

Differential Diagnosis

• Consider alternative diagnoses if maximum disability occurs within 24 hours or after 4 weeks 1
• Rule out other causes of acute weakness: brainstem/spinal cord disorders, toxic neuropathies, metabolic disorders, infections 1

Treatment

Immunotherapy

• Intravenous immunoglobulin (IVIg) at 0.4 g/kg daily for 5 days is recommended for non-ambulant patients within 2 weeks of symptom onset 1, 3, 2
• Plasma exchange (200-250 ml/kg in five sessions over 1-2 weeks) is an effective alternative for patients within 4 weeks of symptom onset 1, 2
• Both treatments are equally effective; choice depends on availability, contraindications, and patient factors 2
• Corticosteroids alone are not recommended for GBS treatment 3, 4
• Combined treatment with PE followed by IVIg is not recommended 2, 4

Respiratory Management

• Monitor respiratory function regularly with vital capacity (risk if <20 ml/kg), maximum inspiratory pressure (risk if <30 cmH₂O), and maximum expiratory pressure (risk if <40 cmH₂O) 3
• Watch for signs of impending respiratory failure: shortness of breath, use of accessory muscles, decreased vital capacity, and inability to count to 20 in one breath 3
• Consider early intubation and mechanical ventilation if respiratory function deteriorates 5

Management of Complications

• Autonomic dysfunction: Monitor heart rate, blood pressure, and manage fluctuations 1
• Pain management: Consider gabapentinoids, tricyclic antidepressants, or carbamazepine 2
• Prevent pressure ulcers, deep vein thrombosis, and hospital-acquired infections 1
• Address psychological symptoms (anxiety, depression, hallucinations) 1
• Monitor swallowing to prevent aspiration 3

Managing Clinical Progression

Treatment Response

• About 40% of patients do not improve within 4 weeks of treatment, which doesn't necessarily indicate treatment ineffectiveness 1
• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1
• For TRFs, repeating the full course of IVIg or switching to plasma exchange is common practice, though evidence is limited 1
• About 5% of patients initially diagnosed with GBS may develop acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) 1, 6

Prognosis

Recovery

• About 80% of patients regain independent walking ability within 6 months 1
• The modified Erasmus GBS outcome score (mEGOS) can predict walking ability 1, 2
• Recovery can continue for >5 years after disease onset 1, 3

Mortality and Complications

• Death occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications 1
• Risk factors for mortality include advanced age and severe disease at onset 1
• Long-term residual complaints include neuropathic pain, weakness, and fatigue 1, 3
• Recurrent episodes of GBS are rare (2-5% of patients) 1

Rehabilitation

• A comprehensive rehabilitation program should include range-of-motion exercises, stationary cycling, walking, and strength training 1, 3
• Intensity of exercise must be closely monitored as overexertion can worsen fatigue 3
• Multidisciplinary approach involving rehabilitation specialists, physiotherapists, occupational therapists, and speech therapists 1, 3

Common Pitfalls and Caveats

• Failure to recognize and treat pain early can significantly impact patient wellbeing 1
• Patients with complete paralysis usually have intact consciousness, vision, and hearing; be mindful of bedside conversations 1
• Vigilance is required during recovery phase as cardiovascular complications can occur even after ICU discharge 1
• Prior GBS is not a strict contraindication for vaccination, but discussion with experts is advised for patients who developed GBS shortly after vaccination 1
• Misdiagnosis of A-CIDP as GBS can lead to inadequate treatment; consider A-CIDP if progression continues beyond 8 weeks 2