Definition

Acute, immune-mediated demyelinating polyneuropathy characterized by an ascending flaccid paralysis and most often preceded by infection

Causes

  • Guillain-Barré Syndrome (GBS) is characterized by axonal degeneration and demyelination, resulting from both cellular and humoral immune responses targeting peripheral nerves
  • An inciting bacterial or viral infection most commonly triggers an immune response that cross-reacts with peripheral nerve axons or myelin sheaths through molecular mimicry (Donofrio 2017)
    • Documented cases of GBS have resulted from a variety of preceding infections, including:
      • Campylobacter jejuni (most common)
      • HIV
      • CMV
      • EBV
      • VZV
      • Zika virus
  • No clear relationship between vaccines and GBS

Epidemiology

  • GBS is relatively rare (100,000 cases worldwide per year) but risk increases with age (Willison et al, 2016)
  • Slightly more common in men than women

Complications

  • Respiratory decompensation 
  • Autonomic dysfunction 
  • Cardiac dysrhythmias
  • Paralysis

Differential Diagnosis

  • CIDP (Chronic Inflammatory Demyelinating Polyneuropathy)
  • ALS (Amyotrophic Lateral Sclerosis)
  • Encephalitis
  • Lyme disease
  • Diabetic neuropathy
  • Metabolic/electrolyte abnormalities
  • Lead poisoning
  • Acute Intermittent Porphyria
  • Thiamine deficiency
  • Polymyositis
  • Cord compression
  • Transverse myelitis
  • Botulism
  • Eaton-Lambert Syndrome
  • Myasthenia gravis

History of Present Illness

  • Rapidly progressive, symmetric weakness in the arms and legs, often occurring distally and in the legs first
  • Neuropathic pain is common and occurs most frequently in the thighs and lower back (Ruts 2010)
  • May have difficulty swallowing or moving facial muscles and/ or eyes if cranial nerve involvement
  • Symptoms progress over days to four weeks, with weakness reaching a nadir at 2-4 weeks
  • History of recent respiratory or gastrointestinal infection, often in the past two weeks
  • Note: one form of GBS, Miller Fisher Syndrome, presents with rapidly evolving ataxia, areflexia, and ophthalmoplegia without weakness due to demyelination and inflammation of cranial nerves II and VI, spinal ganglia, and peripheral nerves

Physical Exam

  • Vital Signs:
    • Autonomic dysfunction occurs in up to 70% of patients, and may present as orthostatic hypotension, hypertension, or tachycardia (Greene-Chandos et al, 2018)
  • Cardiac:
    • Sinus tachycardia is most common (1/3 of patients), but a wide variety of dysrhythmias including bradyarrhythmias can occur (Mukerji et al, 2009)
  • Pulmonary:
    • Respiratory Decompensation: up to 30% of patients will require mechanical ventilation due to profound muscle weakness (Orlikowski et al, 2004)
  • Neurologic:
    • Symmetric bilateral weakness in the arms and legs, distal > proximal
    • Areflexia or hyporeflexia 
    • Oculomotor or bulbar weakness with cranial nerve deficits may occur
    • Mild sensory loss (ie loss of proprioception) is possible (Donofrio, 2017)
  • GU:
    • Urinary retention due to autonomic dysfunction may occur (Greene-Chandos et al, 2018)

Workup

  • Diagnosis is generally made clinically
  • Consider MRI to rule out cord compression if clinically appropriate
  • Electrolytes
  • Complete Blood Count
  • Lumbar puncture should be performed to rule out other diagnoses
    • CSF analysis classically reveals elevated protein with a normal cell count (Fokke et al, 2017), WBCs < 10-50, normal opening pressure
    • Protein elevation may be seen only after several days
    • Consider other diagnoses if WBC > 10-50

ED Management 

  • Up to 1/3 of patients will require intubation and mechanical ventilation for respiratory support (Orlikowski et al, 2004)
    • Do not use succinylcholine for RSI
    • Helpful to obtain forced vital capacity (FVC=  largest volume of gas that a patient can exhale) or negative inspiratory flow (NIF = greatest negative pressure that a patient can generate)
      • No hard and fast value to determine intubation, but FVC < 20 ml/kg or NIF < 30 cm H2O indicate risk of respiratory decompensation; important to trend values as well as evaluate the whole clinical picture (including respiratory rate, oxygenation, etc.)
  • Low threshold for ICU disposition for respiratory monitoring
  • Supportive care of autonomic instability 
  • Neurology consult
  • Definitive treatment (often initiated after hospital admission) involves intravenous immunoglobulin and/or plasmapheresis (Chevret et al, 2017)
  • There is moderate evidence that corticosteroid use in concurrence with IVIG may hasten recovery, but should not be used as monotherapy (Hughes et al, 2016)
  • Complete recovery is common, and typically occurs several weeks to months after initial symptom onset

Take Home Points

  • GBS is an acute, immune-mediated polyneuropathy that is triggered by recent infection, most commonly Campylobacter jejuni
  • Patients commonly present with progressive ascending distal weakness and paralysis, areflexia, neuropathic pain, and autonomic instability
  • LP is useful to rule out other diagnoses – CSF typically reveals elevated protein and normal cell count
  • Up to one third of patients will require intubation and mechanical ventilation due to respiratory weakness; frequent reassessment and clinical monitoring are key

References

Chevret  S, Hughes  RAC, Annane  D. Plasma exchange for Guillain‐Barré syndrome. Cochrane Database of Systematic Reviews (2017) 2:CD001798. DOI: 10.1002/14651858.CD001798.pub3. PMID: 28241090

Donofrio PD. Guillain-Barré Syndrome. Continuum (2017) 23(5):1295–1309 DOI: 10.1212/CON.0000000000000513 PMID: 28968363

Drasler E, Druck J.  Guillan-Barre Syndrome.  Rosen & Barkin’s 5-Minute Emergency Medicine Consult, 2015; 476-477.  

Fokke C, Vandenberg B, Drenthen J, Walgaard C, Vandoorn PA, Jacobs BC. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain (2014) 137:33-43. DOI:     10.1093/brain/awt285. PMID: 24163275

Greene-Chandos D., Torbey, M. Critical Care of Neuromuscular Disorders. Continuum (2018) 24(6, Neurocritical Care): 1753-1775. DOI: 10.1212/CON.0000000000000682 PMID: 30516604

Hughes  RAC, Brassington  R, Gunn  AA, van Doorn  PA. Corticosteroids for Guillain‐Barré syndrome. Cochrane Database of Systematic Reviews (2016) 10: CD001446. DOI: 10.1002/14651858.CD001446.pub5. PMID: PMC6464149

Mukerji S1, Aloka F, Farooq MU, Kassab MY, Abela GS. Cardiovascular complications of the Guillain-Barré syndrome. Am J Cardiol. (2009) 104(10):1452-5. doi: 10.1016/j.amjcard.2009.06.069. PMID: 19892067

Orlikowski, D., Prigent, H., Sharshar, T. et al. Respiratory dysfunction in guillain-barré syndrome Neurocrit Care (2004) 1(4):415-422. DOI: 10.1385/NCC:1:4:415 PMID: 16174943

Ruts L, Drenthen J, Jongen JL, Hop WC, Visser GH, Jacobs BC, Vandoorn PA. Pain in Guillain-Barre syndrome: a long-term follow-up study. Neurology (2010). 75(16):1439-47. doi: 10.1212/WNL.0b013e3181f88345 PMID: 20861454Willison HJ, Jacobs BJ, Vandoorn PA. Guillain-Barré syndrome. The Lancet (2016) 388(10045):717-727. https://doi.org/10.1016/S0140-6736(16)00339-1