Fong Choong Yi, Aung Hnin Wint Wint, Khairani Arie, Gan Chin Seng, Shahrizaila Nortina, Goh Khean Jin
Division of Paediatric Neurology, Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Division of Paediatric Neurology, Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia; Paediatric Neurology Unit, Yangon Children Hospital, Yangon, Myanmar.
Brain Dev. 2018 Jun;40(6):507-511. doi: 10.1016/j.braindev.2018.02.001. Epub 2018 Feb 17.
Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP). He received intravenous pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis with complete clinical recovery after 6 weeks of illness and improved NCS findings from week 16. This is the first case of paediatric BBE with overlapping GBS with an AIDP subtype of GBS. It expands the clinical spectrum of this condition in children. Our case highlights the importance of sequential NCS in paediatric BBE with overlapping GBS for accurate electrophysiological diagnosis and prognosis particularly if the first NCS findings are not informative.
比克斯特法夫脑干脑炎（BBE）是一种罕见的免疫介导性疾病，其特征为眼肌麻痹、共济失调和意识障碍，如果伴有肢体无力，则可能与吉兰-巴雷综合征（GBS）重叠。我们报告了一名7个月大的男孩，最初表现为眼肌麻痹，随后出现四肢迅速进行性麻痹和意识障碍。初步诊断为重叠GBS的BBE。连续神经传导研究（NCS）结果与急性炎症性脱髓鞘性多发性神经病（AIDP）相符，支持了这一诊断。他接受了静脉注射甲基强的松龙冲击治疗、静脉注射免疫球蛋白和血浆置换，发病6周后临床完全康复，第16周时NCS结果有所改善。这是首例重叠GBS且GBS为AIDP亚型的小儿BBE病例。它拓宽了儿童这种疾病的临床谱。我们的病例强调了连续NCS在重叠GBS的小儿BBE中对于准确的电生理诊断和预后的重要性，特别是如果首次NCS结果无诊断价值时。
