Guillain-Barré Syndrome

Guillain-Barré Syndrome is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • A group of acquired autoimmune disorders causing acute peripheral neuropathy and ascending paralysis that progressively worsens for up to 4 weeks followed by a slow spontaneous recovery of function
  • Subtypes classified by pattern of neural injury:
    • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP): progressive limb weakness with areflexia (~95% of GBS cases in Europe and North America)
    • Axonal subtypes:
      • Acute motor axonal neuropathy (AMAN): pure motor neuropathy strongly associated with Campylobacter jejuni and a higher rate of respiratory failure (~5% of cases in Europe and North America but 30–47% of cases in China, Japan, and Central and South America)
      • Acute motor-sensory axonal neuropathy (AMSAN): combined motor–sensory neuropathy; poor prognosis with prolonged course
    • Regional subtypes:
      • Miller Fisher syndrome (MFS): triad with ophthalmoplegia, ataxia, and areflexia; antibodies to GQ1b present in 90% of patients with MFS
      • Bickerstaff encephalitis: possible variant of MFS with encephalopathy, ophthalmoplegia, ataxia, and hyperreflexia
      • Pharyngeal-cervical-brachial GBS: Parasympathetic and cholinergic dysfunction leads to neck, arm, and oropharyngeal weakness along with upper extremity areflexia.
    • Sensory subtypes:
      • Acute pandysautonomia: orthostatic hypotension, gastroparesis, ileus, constipation/diarrhea, sudomotor/pupillary abnormalities, and neuropathic pain
      • Acute sensory ataxic neuropathy (ASAN): controversial variant with sensory loss and ataxia
  • Polyneuritis cranialis: bilateral cranial nerve involvement and severe peripheral sensory loss associated with cytomegalovirus (CMV) infections
  • Synonym(s): GBS, AIDP; Landry-Guillain-Barré-Strohl syndrome, acute inflammatory idiopathic polyneuritis; acute autoimmune neuropathy; Landry ascending paralysis

ALERT
Rapidly progressing paralysis and respiratory failure occur in 20–30% of patients. Some require mechanical ventilation within 48 hours.

Alert
Areflexia is a red flag for GBS in patients with rapidly progressive limb weakness.

Alert
A history of weakness preceded by respiratory or GI infection suggests GBS.

Epidemiology

Incidence
  • Most common acute paralytic disease in Western countries
  • 0.6 to 2.0/100,000 worldwide
  • U.S. incidence: 0.9 to 1.8/100,000
  • Increases with age: 0.8/100,000 in children <18 years of age; 3/100,000 in adults > 60 years
  • 1.5 times higher incidence in males

Etiology and Pathophysiology

  • Autoimmune process targets Schwann cell surface membrane, myelin, and/or gangliosides causing peripheral nerve destruction and demyelination.
  • Pathogenesis thought to involve molecular mimicry (i.e., an immune response to antigenic targets that are coincidentally shared by infectious organisms and host peripheral nerve tissue)

Genetics
Host factors appear to play a role in GBS, but no clear genetic risk has been identified.

Risk Factors

Influenza vaccinations

  • Inactivated seasonal flu vaccines associated with an increase in GBS risk equivalent to one case/million vaccines above background incidence (far less than the 17 cases of GBS per million people infected with influenza virus)
  • Incidence of GBS associated with influenza vaccine has steadily decreased over time.
  • Of historical note: Increased incidence during 1976 U.S. National H1N1 Immunization Program had vaccine-attributable risk of 8.8 per million recipients compared to 1.6 per million recipients in the 2009 H1N1 vaccination campaign.

Commonly Associated Conditions

Infection of the respiratory (22–53%) or GI tract (6–26%) in preceding 6 weeks

  • C. jejuni: most common precipitant of GBS, (21–40% of cases):
    • Associated with axonal degeneration, slower recovery, more severe residual disability
  • CMV: Primary CMV infection precedes 10–22% of cases.
  • Rarely associated with Mycoplasma pneumoniae, influenza infection, Epstein-Barr virus, varicella-zoster virus, HIV infection, and some arboviral infections

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Citation

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TY - ELEC T1 - Guillain-Barré Syndrome ID - 816408 ED - Baldor,Robert A, ED - Domino,Frank J, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816408/all/Guillain_Barré_Syndrome PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -