Nagashima Takahide, Koga Michiaki, Odaka Masaaki, Hirata Koichi, Yuki Nobuhiro
Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321-0293, Japan.
J Neurol Sci. 2004 Apr 15;219(1-2):139-45. doi: 10.1016/j.jns.2004.01.005.
Patients with the pharyngeal-cervical-brachial variant (PCB) of Guillain-Barré syndrome (GBS) have anti-GT1a IgG with or without GQ1b reactivity, whereas those with Fisher syndrome (FS) or Bickerstaff's brainstem encephalitis (BBE) have anti-GQ1b IgG antibodies which cross-react with GT1a. The nosological relationship between these conditions has yet to be established. To investigate the relationships between each manifestation and between clinical features and the coexistence of anti-GQ1b IgG, we reviewed neurological signs present during illnesses of 140 patients who had anti-GT1a IgG. Based on our criteria, FS was diagnosed for 64 (46%) patients, GBS for 22 (16%), BBE for 14 (10%), and PCB for 6 (4%). Overlapping conditions were diagnosed for some patients: FS and GBS (5%), PCB and FS (5%), BBE and GBS (4%), and PCB and BBE (1%). Patients who initially had bulbar palsy developed not only PCB but FS or BBE. The population of anti-GT1a-positive patients frequently had ophthalmoplegia, ataxia, and areflexia, whereas the subpopulation who had anti-GT1a IgG without GQ1b reactivity frequently had preceding diarrhea as well as oropharyngeal, neck, and limb weakness. Patients with anti-GT1a IgG presented a variety of clinical conditions, indicative of a continuous clinical spectrum. A major part of this clinical variation was due to the coexistence of anti-GQ1b IgG. The presence of a common autoantibody (anti-GT1a IgG) and overlapping illnesses suggests that PCB is closely related not only to GBS but to FS and BBE as well.
吉兰-巴雷综合征（GBS）咽颈臂变异型（PCB）患者有抗GT1a IgG，有或无GQ1b反应性，而费舍尔综合征（FS）或比克斯特法夫脑干脑炎（BBE）患者有与GT1a交叉反应的抗GQ1b IgG抗体。这些病症之间的疾病分类关系尚未确定。为了研究每种表现之间以及临床特征与抗GQ1b IgG共存之间的关系，我们回顾了140例有抗GT1a IgG患者疾病期间出现的神经体征。根据我们的标准，64例（46%）患者被诊断为FS，22例（16%）为GBS，14例（10%）为BBE，6例（4%）为PCB。一些患者被诊断为重叠病症：FS和GBS（5%）、PCB和FS（5%）、BBE和GBS（4%）以及PCB和BBE（1%）。最初有延髓麻痹的患者不仅发展为PCB，还发展为FS或BBE。抗GT1a阳性患者群体经常出现眼肌麻痹、共济失调和反射消失，而没有GQ1b反应性的抗GT1a IgG亚群体经常有腹泻前驱症状以及口咽、颈部和肢体无力。有抗GT1a IgG的患者表现出多种临床病症，表明存在连续的临床谱。这种临床变异的主要部分是由于抗GQ1b IgG的共存。共同自身抗体（抗GT1a IgG）的存在和重叠病症表明，PCB不仅与GBS密切相关，而且与FS和BBE也密切相关。
