Ito Hiroto, Hatanaka Yuki, Fukami Yuki, Harada Yumiko, Kobayashi Rei, Okada Hisashi, Uchibori Ayumi, Chiba Atsuro, Okuda Satoshi
Department of Neurology, National Hospital Organization Nagoya Medical Center, 4-1-1 Sannomaru, Naka-ku, Nagoya, Japan.
Department of Neurology, Teikyo University, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
BMC Neurol. 2018 May 23;18(1):72. doi: 10.1186/s12883-018-1077-5.
Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE) are a group of autoimmune neurological disorders (GBS spectrum disorder) that rarely recur. Recently, anti-ganglioside complex antibodies (GSC-Abs) were identified in patients with GBS spectrum disorder. However, there has been no case report describing GSC-Abs profiles in a recurrent case showing different phenotypes.
We report the case of a 33-year-old male patient with GQ1b-seronegative BBE-GBS after two prior episodes of MFS-GBS. Our patient showed ophthalmoplegia, ataxia, areflexia and a weakness of the extremities (MFS and GBS symptoms) in all episodes. In the episode reported here, our patient showed disturbed consciousness and an extensor response to cutaneous plantar stimulation was observed (BBE symptoms), with severe disability and requirement for artificial respiration management. GSC-Abs detected in previous episodes were also detected in the subsequent episodes, while new GSC-Abs emerged in each episode. Interestingly, whereas antibodies to GA1/GQ1b and GA1/GT1a, which are commonly identified in patients with GBS, MFS or BBE, appeared in all episodes, antibodies to GD1a/GD1b and GD1b/GT1b, which are predominantly associated with severe disability and the requirement for artificial respiration management in GBS, emerged for the first time in this episode.
This study reports novel phenomena about the GSC-Abs profiles and its relationship with clinical features in a case with recurrent GBS spectrum disorder, showing different phenotypes in different episodes. Further studies are required to reveal the significance of the GSC-Abs profiles in recurrent GBS spectrum disorder.
吉兰-巴雷综合征（GBS）、米勒-费雪综合征（MFS）和比克斯特法夫脑干脑炎（BBE）是一组很少复发的自身免疫性神经系统疾病（GBS谱系障碍）。最近，在GBS谱系障碍患者中发现了抗神经节苷脂复合抗体（GSC-Abs）。然而，尚无病例报告描述复发性病例中表现出不同表型的GSC-Abs谱。
我们报告了一名33岁男性患者的病例，该患者在先前两次发生MFS-GBS后出现GQ1b血清阴性的BBE-GBS。我们的患者在所有发作中均表现出眼肌麻痹、共济失调、腱反射消失和四肢无力（MFS和GBS症状）。在本报告的发作中，我们的患者出现意识障碍，对足底皮肤刺激表现为伸肌反应（BBE症状），伴有严重残疾且需要人工呼吸管理。先前发作中检测到的GSC-Abs在随后的发作中也被检测到，而每次发作中都出现了新的GSC-Abs。有趣的是，虽然在GBS、MFS或BBE患者中常见的针对GA1/GQ1b和GA1/GT1a的抗体在所有发作中均出现，但在GBS中主要与严重残疾和人工呼吸管理需求相关的针对GD1a/GD1b和GD1b/GT1b的抗体在本次发作中首次出现。
本研究报告了复发性GBS谱系障碍病例中GSC-Abs谱及其与临床特征关系的新现象，该病例在不同发作中表现出不同表型。需要进一步研究以揭示GSC-Abs谱在复发性GBS谱系障碍中的意义。
