7) Guillain-Barré症候群

Recent advance in pathogenesis of Guillain-Barré syndrome

C. jejuni(Campylobacter jejuni)腸炎後に発症する軸索型Guillain-Barré症候群は，分子相同性による抗体産生機序が立証された最初の自己免疫疾患である．患者から分離されたC. jejuniのリポオリゴ糖はヒト神経組織に存在するGM1ガングリオシドと糖鎖相同性を有していた．ヒト組織に類似した構造をもつ病原体に感染することにより病原抗体である抗ガングリオシド抗体が誘導され，神経組織の傷害が起こり弛緩性四肢麻痺に至る．抗ガングリオシド抗体の病的意義の確立は，抗体の種類によるGuillain-Barré症候群とその類縁疾患の分類再編を支持する．

Guillain-Barré syndrome, the most frequent manifestation of which is acute progressive limb weakness, is the first verification of the causative mechanism of molecular mimicry in autoimmune diseases.　IgG antibodies against gangliosides are found in patients with Guillain-Barré syndrome, following Campylobacter jejuni enteritis.　Carbohydrate mimicry was identified between human GM1 ganglioside and the lipo-oligosaccharide of C. jejuni from the patients.　Guillain-Barré syndrome experimental rabbits and mice revealed the mechanism of the induction of pathogenic antibodies against gangliosides.　Understanding of this pathogenesis is helpful in the reclassification of the nosologic positions of Guillain-Barré syndrome and its subtypes.