Ahmed Mushtaq, Jawaid Hafsa, Ali Farhan, Saleem Ayesha, Ejaz Muzamil Shabana
Department of Paediatric Unit III Dr. Ruth KM Pfau Civil Hospital, Karachi, Pakistan.
Final Year MBBS Student, Dow Medical College, Dow University of Health Science, Karachi, Pakistan.
J Pak Med Assoc. 2020 Nov;70(11):2054-2056. doi: 10.5455/JPMA.51328.
Bickerstaff Brainstem Encephalitis, a rare variant of Guillain- Barre Syndrome has an estimated prevalence of eight per 100 million individuals. It presents with the classic triad of ataxia, ophthalmoplegia and altered consciousness. We report the case of a 10-year-old child who presented with low grade fever, hypersomnia and inability to walk. Central nervous system examination revealed ophthalmoplegia and multiple cranial nerve palsies. However, CSF examination showed lack of albuminocytological dissociation with no previous history of respiratory or gastrointestinal tract infection. Unavailability of Anti-GQ1b antibodies led to a diagnosis based on suggestive clinical features, abnormal MRI signals and prompt response to corticosteroid administration. Intravenous Methyl Prednisolone in a dose of 30mg/kg/day was administered for 10 days followed by oral Prednisolone 2 mg/kg/day. After complete recovery the patient was discharged, Prednisolone was tapered gradually and eventually discontinued after four months.
比克斯特费尔德脑干脑炎是吉兰-巴雷综合征的一种罕见变异型，估计每1亿人中约有8例发病。其典型表现为共济失调、眼肌麻痹和意识改变三联征。我们报告了一例10岁儿童病例，该患儿表现为低热、嗜睡和无法行走。中枢神经系统检查发现眼肌麻痹和多组颅神经麻痹。然而，脑脊液检查显示无蛋白细胞分离现象，且既往无呼吸道或胃肠道感染史。由于无法检测到抗GQ1b抗体，故根据提示性临床特征、异常的MRI信号以及对皮质类固醇治疗的迅速反应做出诊断。静脉注射甲泼尼龙，剂量为30mg/kg/天，持续10天，随后口服泼尼松龙2mg/kg/天。完全康复后患者出院，泼尼松龙逐渐减量，最终在四个月后停药。
