Sakai Toshiyuki, Kondo Masahide, Tomimoto Hidekazu, Yamagishi Yuko
Department of Neurology, Saiseikai Matsusaka General Hospital.
Rinsho Shinkeigaku. 2016 Oct 28;56(10):694-697. doi: 10.5692/clinicalneurol.cn-000887.
We report a 28-year-old woman with the overlap of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation. She showed total ophthalmoplegia, dysphagia, dysarthria, upper extremity weakness, cerebellar ataxia, slightly diminished superficial sensations in her hands and feet, urinary retention and constipation 14 days after preceding infection. Laboratory data showed elevations of antiganglioside antibodies to GT1b, GD1b, GQ1b, GD3 and GT1a in the IgG subclass. There was slight elevation of protein with no pleocytosis in cerebrospinal fluid. After administration of intravenous immunoglobulin (IVIg), only the titer of antiganglioside antibody to GQ1b was decreased, and she showed rapid improvement in dysphagia, urinary retention and constipation, and slow recovery in ophthalmoplegia and cerebellar ataxia. The elevations of antiganglioside antibodies to GQ1b may be pathologically related to autonomic involvement such as urinary retention and constipation in that IVIg seems to be effective. The present case suggests that GQ1b may also locate in the autonomic nerve that plays bladder and defecation functions, and that incidence of neurological symptoms and the response of treatment may differ according to each GQ1b localization.
我们报告一例28岁女性,患有费舍尔综合征和吉兰-巴雷综合征咽颈臂型重叠,并伴有尿潴留和便秘。她在先前感染14天后出现完全性眼肌麻痹、吞咽困难、构音障碍、上肢无力、小脑共济失调、手足浅感觉稍减退、尿潴留和便秘。实验室数据显示IgG亚类中抗神经节苷脂抗体GT1b、GD1b、GQ1b、GD3和GT1a升高。脑脊液中蛋白轻度升高,无细胞增多。静脉注射免疫球蛋白(IVIg)后,仅抗神经节苷脂抗体GQ1b的滴度降低,她的吞咽困难、尿潴留和便秘迅速改善,眼肌麻痹和小脑共济失调恢复缓慢。抗神经节苷脂抗体GQ1b的升高可能在病理上与尿潴留和便秘等自主神经受累有关,因为IVIg似乎有效。本病例提示GQ1b也可能位于发挥膀胱和排便功能的自主神经中,并且神经症状的发生率和治疗反应可能因每个GQ1b的定位而异。