Kasuya J, Miyazono T, Takenaga S, Arimura K, Osame M, Kusunoki S
Third Department of Internal Medicine, Kagoshima University School of Medicine.
Rinsho Shinkeigaku. 1999 May;39(5):538-41.
A 49-year-old man presented with hoarseness, dysphagia, muscle atrophy and weakness of deltoid, trapezius, sternocleidomastoid, rhomboid, anterior serratus, infraspinatus and supraspinatus. Anti-Gal-C IgM antibody was positive in the serum. The other antiganglioside antibodies (GM1, GM2, GM3, GD1a, GD1b, GD3, GT1a, GT1b, GQ1b, GA1, GalNAc-GD1a, GM1b) were negative. Patient contracted pneumonia but whether it was due to mycoplasma was not evident. Plasmapheresis improved his clinical state including a decrease of the antibody. This case was diagnosed pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, and anti-Gal-C antibody seemed to be correlated with the pathogenesis of this syndrome. Gal-C is a major glycolipid of myelin and the cell membrane of the myelin-forming cell (oligodendrocytes and Schwann cells) and is free of specific localization and distribution. The mechanism how the anti-Gal-C IgM antibody induced bulbar paralysis and the symptoms localizing neck and upper limbs remains to be known.
一名49岁男性出现声音嘶哑、吞咽困难、肌肉萎缩以及三角肌、斜方肌、胸锁乳突肌、菱形肌、前锯肌、冈下肌和冈上肌无力。血清中抗半乳糖脑苷脂（Gal-C）IgM抗体呈阳性。其他抗神经节苷脂抗体（GM1、GM2、GM3、GD1a、GD1b、GD3、GT1a、GT1b、GQ1b、GA1、N-乙酰半乳糖胺-GD1a、GM1b）均为阴性。患者患了肺炎，但肺炎是否由支原体引起并不明确。血浆置换改善了他的临床状态，包括抗体水平下降。该病例被诊断为格林-巴利综合征的咽-颈-臂型变异型，抗Gal-C抗体似乎与该综合征的发病机制相关。Gal-C是髓鞘以及形成髓鞘的细胞（少突胶质细胞和施万细胞）细胞膜的主要糖脂，且无特定的定位和分布。抗Gal-C IgM抗体如何诱发延髓麻痹以及颈部和上肢定位症状的机制仍有待明确。
