Fisher syndrome or Bickerstaff brainstem encephalitis? Anti-GQ1b IgG antibody syndrome involving both the peripheral and central nervous systems
Fisher syndrome or Bickerstaff brainstem encephalitis? Anti-GQ1b IgG antibody syndrome involving both the peripheral and central nervous systems
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DOI：
10.1002/mus.10246
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发表时间：
2002-12-01
期刊：
影响因子：
3.4
通讯作者：
Yuki, N
中科院分区：
文献类型：
--
作者：
Ogawara, K;Kuwabara, S;Yuki, N
我们描述一位27岁女性，临床表现为Fisher综合征三联征（眼肌麻痹、共济失调和反射消失）、意识障碍、双侧面瘫和偏侧感觉丧失。她的血清抗GQ1b免疫球蛋白G（IgG）抗体阳性。脑电图结果显示（弥漫性慢活动），正中体感诱发电位（缺少皮质N20，颈部N13正常）和眨眼反射研究（R2缺失）提示中枢功能障碍，而面神经传导研究结果（复合肌肉动作电位的低振幅）、F波和H反射研究脑干听觉诱发电位（I波潜伏期延长）提示外周异常。本病例支持Fisher综合征和Bickerstaff脑干脑炎之间的假设连续性。这两种情况可能代表一个单一的自身免疫性疾病介导的抗GQ1b抗体，通常涉及外周和偶尔中枢神经系统。(C)2002 Wiley Periodicals，Inc.
We describe a 27-year-old woman who showed the clinical triad of Fisher syndrome (ophthalmoplegia, ataxia, and areflexia), a disturbance of consciousness, facial diplegia, and hemisensory loss. Her serum was positive for anti-GQ1b immunoglobulin G (IgG) antibody. The electroencephalographic findings (diffuse slow activity), median somatosensory evoked potential (absent cortical N20 with normal cervical N13), and blink reflex studies (absent R2) suggested central dysfunction, whereas results of facial nerve conduction studies (low amplitudes of compound muscle action potentials), F-wave and H-reflex studies (absent F-waves and soleus H-reflexes), and brainstem auditory evoked potentials (prolongation of wave I latency) suggested peripheral abnormalities. This case supports the hypothesized continuity between Fisher syndrome and Bickerstaff brainstem encephalitis. These two conditions may represent a single autoimmune disease mediated by anti-GQ1b antibody, usually involving the peripheral and occasionally the central nervous systems. (C) 2002 Wiley Periodicals, Inc.