Pathogenesis of axonal Guillain-Barré syndrome: hypothesis.

Pathologic studies of acute motor axonal neuropathy show strong evidence of the presence of primary axonal Guillain-Barré syndrome (GBS). The pathogenesis of axonal GBS is speculated to be as follows: (1) Infection by an organism induces the high production of a cross-reactive antibody between an infectious agent and the motor nerve axon in patients with a particular immunogenetic background. (2) The antineural antibody binds to the motor nerve terminals, thereby inhibiting motoneuron excitability and causing muscular weakness. (3) Binding of the antineural antibody, or subsequent functional impairment of the motoneurons, causes the motor axon to degenerate from the terminals. (4) In severe cases, extensive axonal loss and central chromatolysis of the motoneurons occur. These inhibit recovery and lead to a poor functional prognosis.