臨床神経学

第49回日本神経学会総会

<シンポジウム11-2>末梢神経障害の研究―最近の進歩―
免疫関連性ニューロパチー

楠 進

近畿大学医学部神経内科〔〒589-8511 大阪府大阪狭山市大野東377-2〕

Guillain-Barré syndrome (GBS) has two types; acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Recently, a nation-wide retrospective study showed that the rate of AMAN is higher in Japan than in western countries. A prospective study is now in progress. Elevated titers of serum anti-ganglioside antibodies are characteristic of GBS. Complement system has been shown to be involved in the anti-ganglioside antibody-mediated pathogenetic mechanisms. Some GBS patients have antibodies specific to a conformational epitope formed by two different gangliosides. Among such anti-ganglioside complex antibodies, anti-GD1a/GD1b IgG antibodies are shown to be associated with severe GBS requiring artificial ventilation. In contrast, antibodies highly specific to GD1b are associated with GBS with ataxia. Sensory ataxic neuropathy is induced by sensitization of rabbits with GD1b. An apoptotic mechanism has recently been shown to be involved in the pathogenesis of this animal model. Most of the patients with Fisher syndrome have anti-GQ1b IgG antibodies. Recent investigation on anti-ganglioside complex antibody showed that antibodies in Fisher syndrome can be subdivided into the three groups; GQ1b-specific, GQ1b/GM1-specific, and GQ1b/GD1a-specific. Research on antibodies to gangliosides and ganglioside complexes will provide us with a clue to develop a novel treatment of GBS.
Full Text of this Article in Japanese PDF (256K)

(臨床神経, 48:1023−1025, 2008)
key words:ギラン・バレー症候群, 脱髄性ニューロパチー, 軸索障害性ニューロパチー, 抗ガングリオシド抗体, ガングリオシド複合体

(受付日:2008年5月17日)