Rho Young Il
Department of Pediatrics, Chosun University School of Medicine, Gwangju, Korea.
Korean J Pediatr. 2014 Oct;57(10):457-60. doi: 10.3345/kjp.2014.57.10.457. Epub 2014 Oct 31.
A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barré syndrome (GBS). In addition, BBE and Fisher syndrome, which are clinically similar and are both associated with the presence of the immunoglobulin G anti-GQ1b antibody, represent a specific autoimmune disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia. A 2-year-old boy presented with rapidly progressive ophthalmoplegia, ataxia, hyporeflexia, weakness of the lower extremities, and, subsequently, disturbance of consciousness. He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.
巴洛综合征(BBE)中的弛缓性四肢轻瘫被认为是重叠性吉兰-巴雷综合征(GBS)的一个体征。此外,BBE和费舍尔综合征在临床上相似,且均与抗GQ1b免疫球蛋白G抗体的存在有关,它们代表一种具有广泛症状谱的特定自身免疫性疾病,这些症状包括眼肌麻痹和共济失调。一名2岁男孩出现快速进展的眼肌麻痹、共济失调、反射减退、下肢无力,随后出现意识障碍。他曾患支气管炎并伴有水样腹泻,实验室检查有近期感染爱泼斯坦-巴尔病毒(EBV)的证据。他被诊断为与EBV相关的重叠性GBS和BBE,并接受了免疫球蛋白和甲泼尼龙联合治疗以及阿昔洛韦治疗,3个月后完全康复。此外,在过去一年中他没有出现任何复发情况。我们认为,中枢性病变(意识障碍)和周围性病变(眼肌麻痹、面部无力、肢体无力和反射消失)的症状和体征组合支持重叠性GBS和BBE的诊断,有助于早期诊断以及进行适当治疗。