Guillain-barré syndrome

Learning objectives

Guillain-Barré syndrome (GBS) is an acute demyelinating polyneuropathy typically occurring as an autoimmune response following a gastrointestinal or respiratory infection
Most common cause of acute, flaccid, neuromuscular paralysis in the United States
Potentially severely debilitating disorder
Mortality rate ~10%
Initial presentation is often misdiagnosed as hysteria

Affects all ages, but a tendency towards young adults and the elderly
Slightly more prevalent in men
Children are less severely affected
Usually occurs within a month of a respiratory or gastrointestinal infection
Common pathogens causing GBS:
- Campylobacter jejuni (associated with axonal degeneration in addition to primary demyelination)
- Epstein Barr virus
- Mycoplasma pneumonia
- Cytomegalovirus

Clinical signs
- Acute inflammatory demyelinating polyradiculopathy
- Acute motor axonal neuropathy
- Acute motor sensory axonal neuropathy
- Miller Fisher syndrome (ataxia, areflexia, and ophthalmoplegia, possibly with limb weakness, ptosis, and facial and bulbar palsy)
Symptoms
- Progressive motor weakness, usually ascending from the legs (more proximal than distal)
- Areflexia
- Facial palsy and bulbar weakness
- Ophthalmoplegia
- Sensory symptoms
- Severe pain, often affecting the girdle area
- Weakness of the respiratory musculature leading to respiratory failure
- Autonomic dysfunction causing under- or overactivity of the sympathetic and parasympathetic systems leading to arrhythmias, fluctuations in blood pressure and pulse, urinary retention, ileus, and excessive sweating

Supportive therapy
- Physiotherapy and occupational therapy
- Counseling
- Nutritional support
- Analgesia
- Thromboembolic prophylaxis
- Respiratory support
  - Indications for intubation and ventilation:
    - Vital capacity <20 mL/kg
    - Maximal inspiratory pressure (MIP) <30 cmH2O
    - Maximal expiratory pressure (MEP) <40 cmH2O
    - Decrease of >30% in vital capacity, MIP or MEP
Specific therapy
- Treatment of choice: IV immunoglobins (0.4 mg/kg daily, 5-6 days)
- Plasmapheresis, up to 5 exchanges of 250 mL/kg of plasma with 4.5% human albumin solution (more difficult to administer, more side-effects and contraindications)
- CSF filtration (rarely performed)

Preoperative
- Many patients are ventilated in the ICU
- Assess bulbar and ventilatory function to predict the need for postoperative ventilation
- Ileus increases the risks of aspiration
Induction
- Rapid sequence induction
- Succinylcholine is contraindicated due to potentially fatal hyperkalemia
- Rocuronium is a suitable alternative
- Autonomic dysfunction can complicate induction and intubation by resulting in a labile pulse and blood pressure
Intraoperative
- Controlled ventilation if respiratory function is impaired
- Avoid nondepolarizing neuromuscular blockers
- Consider extubating when fully recovered or once bulbar reflexes have returned
Postoperative
- Ventilation is often required
- Careful monitoring of respiratory function
- Adequate analgesia