Bickerstaff Brainstem Encephalitis and Miller-Fisher Syndrome Overlap Following Stenting of Vertebral Artery: A Case Report

doi:10.3969/j.issn.1673-5765.2023.08.010

Abstract

Abstract: Miller-Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE) are extremely rare in clinical practice. In recent years, MFS together with Guillan-Barré syndrome (GBS) or BBE has been reported abroad sporadically, possibly affecting the peripheral and/or central nervous systems, with monophasic symptoms. However, the frequency of overlapping clinical symptoms and signs of these three diseases is very low, and there was no report in China so far. Therefore, this paper described a 64-year-old male who presented with subacute onset, bilateral symmetrical weakness of limbs, paralysis of extraocular muscles, disappearance of tendon reflexes of both lower limbs, accompanied by drowsiness and other disturbances of consciousness after vertebral artery stenting surgery. The course of disease peaked at 2 weeks. The results of electromyography suggested neurogenic damage, and the finding of cerebrospinal fluid (CSF) and serological results showed a significant increase in protein but a decrease in cell count. It was supported by positivity for anti-GQ1b IgG antibody (GQ1b-IgG) and anti-GT1a IgG antibody (GT1a-IgG) in serum and CSF. Thus, this patient was diagnosed as MFS overlapping BBE. The results of this paper emphasizes that MFS, BBE, and GBS may be different subtypes of the same disease, and GQ1b-IgG and GT1a-IgG further reveal the common immune related pathophysiological mechanisms of these disease subtypes. It prompts clinicians to consider the presence of GQ1b-IgG syndrome and give immunoglobulin therapy as soon as possible to improve the patients’ prognosis when they presented with the overlapping and cross clinical manifestations of MFS, BBE, and GBS, especially involved peripheral and central nervous systems.

Key words: Miller-Fisher syndrome; Autoimmune disease; Vertebral artery; Peripheral neuropathy; Anti-ganglioside antibody

摘要： Miller-Fisher综合征（Miller-Fisher syndrome，MFS）和Bickerstaff脑干脑炎（Bickerstaff brainstem encephalitis，BBE）临床较为罕见。近两年，国外可见散在病例报告MFS叠加格林巴利综合征（Guillan-Barré syndrome，GBS）或BBE，共同影响外周和（或）中枢神经系统，并伴有单相症状。然而，三种疾病叠加的临床症状或体征十分罕见，迄今为止国内未见报道。因此，本研究报告1例64岁男性椎动脉术后亚急性起病，表现为典型的对称性四肢无力、眼外肌麻痹、双下肢腱反射消失，同时伴有嗜睡等意识障碍表现，病程在2周时到达高峰。肌电图检查结果呈神经源性损害，脑脊液和血清学结果提示蛋白-细胞分离现象，GQ1b抗体（anti-GQ1b IgG antibody，GQ1b-IgG）和GT1a抗体（anti-GT1a IgG antibody，GT1a-IgG）阳性，临床确诊为MFS叠加BBE。本研究结果强调了MFS、BBE、GBS可能是同一疾病的不同亚型，GQ1b和GT1a抗体进一步揭示了这些疾病亚型的共同免疫相关病理生理机制。临床上，遇到MFS、BBE和GBS叠加、交叉的临床表现，周围和中枢神经系统同时受累时，要考虑到GQ1b抗体综合征可能，尽快给予免疫球蛋白治疗以改善患者预后。

关键词: 米-费综合征; 自身免疫性疾病; 椎动脉; 周围神经病; 抗神经节苷脂抗体

JIANG Jiwei, LI Zixiao, DONG Kehui, PU Yuehua, XU Jun, GONG Xiping. Bickerstaff Brainstem Encephalitis and Miller-Fisher Syndrome Overlap Following Stenting of Vertebral Artery: A Case Report[J]. Chinese Journal of Stroke, 2023, 18(8): 934-939.

姜季委, 李子孝, 董可辉, 濮月华, 徐俊, 龚浠平. 椎动脉支架术后Miller-Fisher综合征叠加Bickerstaff脑干脑炎1例[J]. 中国卒中杂志, 2023, 18(8): 934-939.

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