Axonal Guillain-Barré syndrome: concepts and controversies.
Acute motor axonal neuropathy (AMAN) is a pure motor axonal subtype of Guillain-Barré syndrome (GBS) that was identified in the late 1990s. In Asia and Central and South America, it is the major subtype of GBS, seen in 30-65% of patients. AMAN progresses more rapidly and has an earlier peak than demyelinating GBS; tendon reflexes are relatively preserved or even exaggerated, and autonomic dysfunction is rare. One of the main causes is molecular mimicry of human gangliosides by Campylobacter jejuni lipo-oligosaccharides. In addition to axonal degeneration, electrophysiology shows rapidly reversible nerve conduction blockade or slowing, presumably due to pathological changes at the nodes or paranodes. Autoantibodies that bind to GM1 or GD1a gangliosides at the nodes of Ranvier activate complement and disrupt sodium-channel clusters and axoglial junctions, which leads to nerve conduction failure and muscle weakness. Improved understanding of the disease mechanism and pathophysiology might lead to new treatment options and improve the outlook for patients with AMAN.
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan. Electronic address: kuwabara-s@faculty.chiba-u.jp.
Satoshi
S
Kuwabara
Nobuhiro
N
Yuki
D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D001369:Axons / Q000276:immunology / Q000502:physiology*; D002169:Campylobacter Infections / Q000150:complications / Q000276:immunology; D016123:Campylobacter jejuni / Q000276:immunology; D003167:Complement Activation; D003937:Diagnosis, Differential; D004195:Disease Models, Animal; D004568:Electrodiagnosis / Q000379:methods; D005677:G(M1) Ganglioside / Q000031:analogs & derivatives / Q000276:immunology; D014943:Global Health; D020275:Guillain-Barre Syndrome / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D018716:Molecular Mimicry; D009046:Motor Neurons / Q000473:pathology; D009431:Neural Conduction; D010951:Plasma Exchange; D016032:Randomized Controlled Trials as Topic; D011901:Ranvier's Nodes / Q000276:immunology; D012021:Reflex, Abnormal; D015222:Sodium Channels / Q000502:physiology
D001323:Autoantibodies; D001324:Autoantigens; D016756:Immunoglobulins, Intravenous; D015222:Sodium Channels; C064020:ganglioside GD1alpha; D005677:G(M1) Ganglioside