Ogawara Kazue, Kuwabara Satoshi, Yuki Nobuhiro
Department of Neurology, Chiba University School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan.
Muscle Nerve. 2002 Dec;26(6):845-9. doi: 10.1002/mus.10246.
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.
我们描述了一名27岁女性，她表现出费舍尔综合征的临床三联征（眼肌麻痹、共济失调和无反射）、意识障碍、双侧面瘫和半身感觉丧失。她的血清抗GQ1b免疫球蛋白G（IgG）抗体呈阳性。脑电图结果（弥漫性慢活动）、正中体感诱发电位（皮质N20缺失而颈段N13正常）和瞬目反射研究（R2缺失）提示中枢功能障碍，而面神经传导研究结果（复合肌肉动作电位波幅降低）、F波和H反射研究（F波和比目鱼肌H反射缺失）以及脑干听觉诱发电位（波I潜伏期延长）提示周围神经异常。该病例支持费舍尔综合征与比克斯特法夫脑干脑炎之间的假设连续性。这两种情况可能代表由抗GQ1b抗体介导的单一自身免疫性疾病，通常累及周围神经系统，偶尔累及中枢神经系统。
