ABSTRACT
The Guillain-Barré syndrome (GBS) represents the prototypic immune-mediated peripheral
neuropathy, which is now recognized as a group of conditions with diverse pathology
and pathogenesis. The observation that plasma exchange and intravenous immunoglobulin
result in clinical improvement, the presence of circulating antibodies targeting structures
on peripheral nerve tissue in sera from GBS patients, and the deposition of immunoglobulins
and complement demonstrated on myelinated fibers in nerve biopsies from affected patients
point to a critical role of the humoral immune response in the pathogenesis of GBS.
However, the observation of inflammatory infiltrates in nerve and the critical pathogenic
role of neuritogenic T lymphocytes as demonstrated in the animal model support the
concept that a disordered cellular immunity is also of critical importance in the
pathogenesis of this neuropathy. Current treatment strategies are aimed at mitigating
the harmful effects of the immune system on peripheral nerve. This review will address
the rationale for immunotherapy in GBS based on experimental and immunologic studies
of the pathogenesis of this disease.
KEYWORDS
Guillain-Barré - syndrome - plasma exchange - intravenous immunoglobulins - molecular
mimicry