Guillain-Barre Syndrome

Background

• Guillain-Barre syndrome (GBS) is a rare autoimmune acute polyradiculoneuropathy with potentially severe symptoms, usually presenting with bilateral weakness starting in the distal lower limbs and spreading proximally and to include the upper limbs.
  • Onset usually follows infection or another immune-stimulating event, with an interval of about 1-4 weeks.
    • Campylobacter jejuni infection is the most commonly reported preceding infection.
    • Other infections, including COVID-19 (novel coronavirus), have also been reported to precede GBS.
  • Symptoms progress over 2-4 weeks to potentially life-threatening severity requiring mechanical ventilation before improving (particularly if mild symptoms) or plateauing for weeks-to-months.
• The annual incidence is 0.8-1.9 cases/100,000 persons, and it increases with advancing age.
• Most patients recover most of their function, but persistent symptoms may include residual pain and fatigue.
• Mortality is 3%-7%.
• History of Guillain-Barre syndrome is not a contraindication to COVID-19 vaccines currently authorized in the United States (Centers for Disease Control and Prevention interim clinical considerations for COVID-19 vaccines [CDC 2021 Mar]).

Evaluation

• Ask patient about limb weakness and sensory symptoms - if they're symmetrical, when they started, and if they got progressively worse over the past few days or weeks.
• Ask about respiratory or gastrointestinal tract infection, or other potential immune-stimulating events, 1-4 weeks before symptom onset.
• Evaluate tendon reflexes, for signs of limb weakness and sensory abnormalities, and signs of autonomic dysfunction.
• Assess pulmonary function with forced vital capacity and maximal expiratory pressure and frequently monitor while Guillain-Barre syndrome (GBS) is a potential diagnosis, as symptom progression to respiratory failure reported in about 30% of patients.
• Consider blood tests or other diagnostic testing based on other suspected conditions.
• Suspect GBS in patients with weakness in legs and arms (usually symmetric and may initially be only in legs) that progresses over several days-to-4 weeks and then plateaus, impaired tendon reflexes in weak limbs, infection or other immune-stimulating event within past 4 weeks, and other causes of symptoms ruled out.
• Cerebrospinal fluid analysis and nerve conduction studies may support diagnosis but are not required for diagnosis.

Management

• Hospital admission required while symptoms progress (usually 2-4 weeks); ensure timely transfer to intensive care unit (ICU) as indicated, and consider early admission to ICU if strong suspicion of future respiratory failure.
• Assess risk of respiratory failure and disability to guide monitoring and management strategies.
• Carefully monitor for respiratory failure (reported in about 30% of patients):
  • Indications for intubation in patients with Guillain-Barre syndrome include any of the following:
    • forced vital capacity < 15 mL/kg of age-adjusted ideal body weight.
    • negative inspiratory force < 60 cm H₂O.
    • hypercapnia (partial pressure of arterial CO₂ > 6.4 kilopascal [48 mm Hg]).
    • hypoxemia (partial pressure of arterial O₂ on ambient air < 7.5 kilopascal [56 mm Hg]).
    • ≥ 2 of inefficient cough, impaired swallowing, and atelectasis.
  • Note that hypercapnia and hypoxemia are generally late findings of respiratory failure. Measure pulmonary function frequently to assess need for intubation.
• Carefully monitor for other potentially life-threatening manifestations such as cardiac arrhythmias, dysphagia, hemodynamic instability, and ileus.
• Also monitor for gastrointestinal and bladder dysfunction and pulmonary and urinary tract infections.
• Consider prophylaxis for deep vein thrombosis and decubitus ulcers.
• Manage pain (reported in most patients).
• Consider starting physical therapy, occupational therapy, and psychosocial therapy while in hospital.
• Start IV immunoglobulin (IVIG) or plasmapheresis (not both) as soon as possible after diagnosis. (Strong recommendation)
  • IVIG regimen - give total dose 2 g/kg as either 0.4 g/kg IV once daily for 5 days, or 1 g/kg IV once daily for 2 days
  • Plasmapheresis regimen - consists of 5 sessions, each comprising 2-3 L of plasma according to body weight, over 2 weeks (2 sessions may show improvement in patients who are able to walk). Perform less frequently than every day to reduce shifts in fluid balance.
• Do not give corticosteroids to treat GBS. (Strong recommendation)
• If 1 treatment-related fluctuation (worsening of symptoms after period of stability or improvement with treatment), consider starting a second course of IVIG or plasmapheresis.
• If > 1 treatment-related fluctuation, suspect alternate diagnosis of acute onset chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).