Guillain-Barré Syndrome

Guillain-Barré Syndrome is a topic covered in the Washington Manual of Medical Therapeutics.

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General Principles

Definition

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy syndrome and a common cause of acute flaccid paralysis. There are many GBS subtypes with marked geographic variability in their prevalence. The clinical syndrome is classically characterized by ascending weakness, distal paresthesias, and areflexia. Classically, GBS follows a viral infection, vaccination, or surgery, but in many instances, no prodrome is identified.

Classification

  • The acute immune/inflammatory demyelinating polyneuropathy (AIDP) variant is the most common GBS variant in North America. It is an acute immune-mediated polyneuropathy/radiculopathy with presumed autoantibodies (as yet unidentified) directed against myelin antigens.
  • Axonal variants of GBS include acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), acute motor conduction block neuropathy (AMCBN), Miller Fisher syndrome (MFS), and pharyngeal-cervical-brachial (PCB) weakness. Many of these syndromes are associated with antiganglioside antibodies directed against gangliosides located in the axolemma near the nodes of Ranvier. These axonal variants are much more common in Japan, China, and third-world countries than they are in the United States. Antibody associations include the following:
    • IgG anti-GM1 antibodies in AMAN and AMCBN
    • IgG anti-GQ1b and less commonly IgG anti-GT1a in MFS, a syndrome consisting of ophthalmoparesis, ataxia, and areflexia
    • IgG anti-GT1a and less commonly IgG anti-GQ1b in PCB weakness syndrome

Pathophysiology

  • GBS results from an attack of peripheral myelin or axons mediated by autoantibodies originally generated in response to an infection that typically precedes the onset of neuropathy symptoms by days to weeks. These antibodies cross-react with the myelin or nodal axolemma antigens via molecular mimicry.
  • This concept is well established in the axonal variants where there is definitive evidence of molecular mimicry between Campylobacter jejuni lipo-oligosaccharides and the ganglioside antigens listed above. In fact, GBS was the first disease to illustrate the concept of molecular mimicry and fulfills all four criteria (“Witebsky postulates”) for designation as an autoimmune disease.
  • This concept is less well established in AIDP, given the absence of a known autoantibody/myelin antigen. Prodromal infections commonly associated with AIDP include cytomegalovirus and Epstein–Barr virus.

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General Principles

Definition

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy syndrome and a common cause of acute flaccid paralysis. There are many GBS subtypes with marked geographic variability in their prevalence. The clinical syndrome is classically characterized by ascending weakness, distal paresthesias, and areflexia. Classically, GBS follows a viral infection, vaccination, or surgery, but in many instances, no prodrome is identified.

Classification

  • The acute immune/inflammatory demyelinating polyneuropathy (AIDP) variant is the most common GBS variant in North America. It is an acute immune-mediated polyneuropathy/radiculopathy with presumed autoantibodies (as yet unidentified) directed against myelin antigens.
  • Axonal variants of GBS include acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), acute motor conduction block neuropathy (AMCBN), Miller Fisher syndrome (MFS), and pharyngeal-cervical-brachial (PCB) weakness. Many of these syndromes are associated with antiganglioside antibodies directed against gangliosides located in the axolemma near the nodes of Ranvier. These axonal variants are much more common in Japan, China, and third-world countries than they are in the United States. Antibody associations include the following:
    • IgG anti-GM1 antibodies in AMAN and AMCBN
    • IgG anti-GQ1b and less commonly IgG anti-GT1a in MFS, a syndrome consisting of ophthalmoparesis, ataxia, and areflexia
    • IgG anti-GT1a and less commonly IgG anti-GQ1b in PCB weakness syndrome

Pathophysiology

  • GBS results from an attack of peripheral myelin or axons mediated by autoantibodies originally generated in response to an infection that typically precedes the onset of neuropathy symptoms by days to weeks. These antibodies cross-react with the myelin or nodal axolemma antigens via molecular mimicry.
  • This concept is well established in the axonal variants where there is definitive evidence of molecular mimicry between Campylobacter jejuni lipo-oligosaccharides and the ganglioside antigens listed above. In fact, GBS was the first disease to illustrate the concept of molecular mimicry and fulfills all four criteria (“Witebsky postulates”) for designation as an autoimmune disease.
  • This concept is less well established in AIDP, given the absence of a known autoantibody/myelin antigen. Prodromal infections commonly associated with AIDP include cytomegalovirus and Epstein–Barr virus.

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