Guillain-Barre Syndrome (GBS)

(aka Acute Inflammatory Demyelinating Polyneuropathy, AIDP)

Epidemiology

  • Incidence
    • Approximately 3500 cases per year in USA and Canada
    • GBS is the most common etiology of acute paralysis with neuromuscular ventilatory failure presenting to acute care hospitals
  • Age: typically occurs in younger patient group than chronic inflammatory demyelinating polyneuropathy (CIDP) (see Chronic Inflammatory Demyelinating Polyneuropathy, [[Chronic Inflammatory Demyelinating Polyneuropathy]])
  • Association with Infection
    • GBS follows a viral infection by 1-3 weeks in 66% of cases
    • Preceding upper respiratory illness or gastrointestinal illness about 1 month before presentation is common

Predisposing Factors

Infection/Vaccination

  • Campylobacter Jejuni Enteritis (see Campylobacter Jejuni, [[Campylobacter Jejuni]]): 26% of cases have positive stool cultures or positive serology (IgA, IgM, and IgG) for C. Jejuni
  • Cytomegalovirus (CMV) (see Cytomegalovirus, [[Cytomegalovirus]])
  • Epstein-Barr Virus (EBV) (see Epstein-Barr Virus, [[Epstein-Barr Virus]])
  • Human Immunodeficiency Virus (HIV) (see Human Immunodeficiency Virus, [[Human Immunodeficiency Virus]])
  • Mycoplasma (se Mycoplasma, [[Mycoplasma]])
  • Post-Swine Flu Vaccination: 500 cases reported in 1976
    • Incubation Period: 1-6 weeks
  • Vaccinia Virus (see Vaccinia Virus, [[Vaccinia Virus]])

Hematologic Disease

Other

  • Post-Surgery (5-10% of cases): occurs within 1-4 weeks
  • Systemic Lupus Eryhthematosus (SLE) (see Systemic Lupus Eryhthematosus, [[Systemic Lupus Eryhthematosus]])

Physiology

  • Acquired demyelinating polyneuropathy (probably immune)
  • Hypoventilation leads to hypoxia/ acidosis -> pulmonary vasoconstriction

Diagnosis

  • ABG
    • Early: hypoxemia (with elevated A-a gradient), hypocapnia
    • Later: hypoxemia/hypercapnia (occurs when VC falls to <33% of normal)
  • PFT’s: restrictive pattern
    • Decreased VC (typically 40-70% predicted/occurs due to muscle weakness, decreased lung compliance, and decreased chest wall compliance)
      • VC <20 ml/kg, MIP < -30 cm H20, and MEP <40 cm H20 (or a decrease at least 30% from initial values in any of these) are correlated with the need for mechanical ventilation [MEDLINE]
    • Decreased TLC
    • Increased RV: due to inability to overcome recoil of chest wall outward at volumes below FRC
    • Decreased MVV
    • Decreased MIP/MEP
      • VC <20 ml/kg, MIP < -30 cm H20, and MEP <40 cm H20 (or a decrease at least 30% from initial values in any of these) are correlated with the need for mechanical ventilation [MEDLINE]
    • Decreased Lung Compliance: due to chronically low lung volumes, microatelectasis, reduced surface tension, and altered interstitial elastic fibers
    • Transdiaphragmatic Pressure: using NG balloon (Pga-Pes)/ normal change >25 cm H2O (referenced to TLC), usually 2-20 cm H2O in bilateral paralysis
  • CXR: may demonstrate aspiration pneumonia or low lung volumes
  • Lumbar Puncture (LP): normal cell count with elevated total protein
    • Elevated protein occurs by end of first week of symptoms
  • EMG/NCV: predominantly demyelinating polyneuropathy
    • Decreased NCV’s in motor and sensory nerves
    • Evidence of demyelination is more common in patients who will require mechanical ventilation (85% of cases) vs those who did not (51% of cases) [MEDLINE]
  • Diaphragmatic EMG/NCV: NCV abnormal at time of maximal weakness, but may be normal early (may help to rule in neuropathy and rule out myopathy and anterior horn cell disease)
  • IgG Anti-GQ1b Antibodies: predicts the need for mechanical ventilation [MEDLINE]

Clinical Manifestations

Neurologic Manifestations

  • Acute Progressive, Ascending Motor Paralysis
    • Limb/truncal muscle weakness
    • Difficult to differentiate from CIDP on clinical symptoms and signs alone (however, onset of polyneuropathy symptoms usually precedes the onset of respiratory failure)
  • Mild Sensory Deficits
  • Dysarthria
  • Areflexia

Pulmonary Manifestations

Other Manifestations

  • Dysphagia (see Dysphagia, [[Dysphagia]])
  • Decubitus Ulcers
  • Impaired Nutrition

Treatment

  • Plasmapheresis
    • Decreases hospital stay and duration of mechanical ventilation
    • Most effective when used in the first 2 weeks of illness
    • Not shown to be of benefit in children
    • Cannot be used in patients with hemodynamic instability (such as recent MI/angina/cardiovascular dysautonomia associated with GBS)
  • Intravenous Immunoglobulin (IVIG): may be effective (however, more trials are required)
  • Mechanical Ventilation: 33% of cases require mechanical ventilation
    • Predictors of Requirement for Mechanical Ventilation
      • VC and MIP/MEP (these are the best tests to follow serially): VC <20 ml/kg, MIP < -30 cm H20, and MEP <40 cm H20 (or a decrease at least 30% from initial values in any of these) are correlated with the need for mechanical ventilation [MEDLINE]
      • EMG/NCV Evidence of Demyelination: more common in patients who will require mechanical ventilation (present in 85% of cases) vs those who did not require mechanical ventilation (present in 51% of cases) [MEDLINE]
      • Presence of Cranial Nerve involvement + IgG Anti-GQ1b Antibodies: predicts the need for mechanical ventilation [MEDLINE]
      • Average Duration of Mechanical Ventilation: 58 days (range: 10-104 days)
      • Early Tracheostomy: should consider due to usual prolonged course of ventilation required
  • Corticosteroids: probably not effective
  • Anti-T-cell Antibody: not effective in decreasing disease severity

Prognosis

  • Mortality Rate: 3-8%
  • Near Complete Recovery Rate: 85% of cases make near complete recovery
    • However, 5-10% of survivors remain serious disabled
  • More severe axonal disease may be predicted by presence of a preceding upper respiratory or gastrointestinal illness

References

  • Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol. 2001 Jun;58(6):893-8 [MEDLINE]
  • Anti-GQ1b antibody as a factor predictive of mechanical ventilation in Guillain-Barré syndrome. Neurology. 2004 Mar 9;62(5):821-4 [MEDLINE]
  • Guillain–Barre syndrome associated with rapid immune reconstitution following allogeneic hematopoietic stem cell transplantation. Bone Marrow Transplantation (2006) 37, 617–619 [MEDLINE]
  • Clinical and electrophysiological predictors of respiratory failure in Guillain-Barré syndrome. Lancet Neurol. 2006 Dec;5(12):1021-8 [MEDLINE]