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

Guillain-Barré Syndrome: Symptoms and Treatment

Guillain-Barré syndrome (GBS) is characterized by rapidly progressive bilateral weakness typically starting in the legs and ascending to the arms and cranial muscles, accompanied by decreased or absent reflexes, with most patients reaching maximum disability within 2 weeks of onset. 1, 2, 3

Clinical Presentation

Core Symptoms

• Bilateral ascending weakness is the hallmark feature, typically starting in the legs and progressing upward to arms and cranial muscles 1, 4
• Diminished or absent deep tendon reflexes, especially in the lower limbs 1, 3
• Distal paresthesias or sensory loss often preceding or accompanying weakness 2, 3
• Back and limb pain is an early symptom in approximately two-thirds of patients 2, 4
• History of recent infection within 6 weeks in about two-thirds of patients 2, 3

Additional Clinical Features

• Dysautonomia including blood pressure fluctuations, heart rate instability, pupillary dysfunction, and bowel/bladder dysfunction 1, 4
• Respiratory muscle weakness leading to respiratory failure in about 20% of patients 1, 5
• Cranial nerve involvement affecting facial, bulbar, and oculomotor functions 4, 6
• Disease progression typically reaches maximum severity within 2 weeks 1, 3

Clinical Variants

• Classic sensorimotor GBS (30-85% of cases): Rapidly progressive symmetrical weakness with sensory signs 2
• Pure motor variant (5-70% of cases): Motor weakness without sensory involvement 2
• Miller Fisher syndrome (5-25% of cases): Characterized by the triad of ophthalmoplegia, ataxia, and areflexia 1, 2

Diagnostic Evaluation

• Neurological examination showing progressive bilateral weakness and reduced/absent reflexes 3, 5
• Cerebrospinal fluid analysis typically shows albumino-cytological dissociation (elevated protein with normal cell count), though this may be normal early in disease course 1, 3
• Electrodiagnostic studies to distinguish between subtypes: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN) 1, 4
• MRI or ultrasound imaging should be considered in atypical cases to rule out other causes 4, 3

Treatment

First-Line Therapies

• Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 days (total dose 2 g/kg) for patients unable to walk unaided within 2-4 weeks of symptom onset 2, 4, 7
• Plasma exchange (PE) is an equally effective alternative treatment (200-250 ml/kg in 4-5 exchanges over 1-2 weeks) 2, 7
• Treatment should be initiated as early as possible in the disease course 4, 8

Important Treatment Considerations

• Corticosteroids alone are not recommended for GBS treatment 4, 7
• For immune checkpoint inhibitor-related GBS, a trial of corticosteroids may be reasonable (methylprednisolone 2-4 mg/kg/day) 1, 4
• About 10% of patients experience treatment-related fluctuations requiring repeated IVIg treatment 8

Monitoring and Supportive Care

• Regular assessment of respiratory function is critical as up to 30% of patients develop respiratory failure 4, 5
• Use the "20/30/40 rule" to assess risk of respiratory failure: vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 4
• Monitor for autonomic dysfunction via ECG, heart rate, blood pressure, and bowel/bladder function 1, 4
• Pain management may include gabapentinoids, tricyclic antidepressants, or carbamazepine 4, 7
• Multidisciplinary supportive care including physiotherapy, occupational therapy, and speech therapy 4, 8

Prognosis

• About 60-80% of patients are able to walk independently 6 months after disease onset 1, 2
• Recovery can continue for more than 3 years after onset 2, 8
• Mortality rate is estimated at 3-10% even with the best medical care 1, 8
• Recurrence is rare, occurring in only 2-5% of cases 2, 8
• About 5% of patients initially diagnosed with GBS turn out to have chronic inflammatory demyelinating polyradiculoneuropathy with acute onset (A-CIDP) 7, 8

Complications

• Respiratory failure requiring mechanical ventilation in about 20-25% of patients 1, 8
• Cardiovascular complications due to autonomic dysfunction 1, 4
• Pain, fatigue, and other residual symptoms may persist for months or years 4, 8
• Pressure ulcers, hospital-acquired infections, and deep vein thrombosis are common complications during hospitalization 4