Department of Medicine, Hospital Pulau Pinang, Georgetown, Pulau Pinang, Malaysia.
Department of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Cheras, Kuala Lumpur, Malaysia.
BMJ Case Rep. 2021 May 24;14(5):e242090. doi: 10.1136/bcr-2021-242090.
Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune encephalitis characterised by ataxia, ophthalmoplegia and altered consciousness. An overlap between BBE with Guillain-Barré syndrome (GBS) shows similar clinical and immunological features. We report a case of BBE with GBS overlap secondary to infection. The triad of altered consciousness, ataxia and ophthalmoplegia were present in the patient. The investigations included cerebrospinal fluid cytoalbuminological dissociation, nerve conduction test that showed prolonged or absent F wave latencies, hyperintensity in the left occipital region on brain MRI and diffuse slow activity on the electroencephalogram. The chlamydia serology was positive indicating a postinfectious cause of BBE syndrome. He required artificial ventilation as his consciousness level deteriorated with tetraparesis, oropharyngeal and respiratory muscle weakness. Immunotherapy with intravenous immunoglobulin and methylprednisolone was commenced. He made good recovery with the treatment. Prompt recognition of this rare condition following chlamydia infection is important to guide the management.
比克法斯特脑干脑炎(BBE)是一种罕见的自身免疫性脑炎,其特征为共济失调、眼肌麻痹和意识改变。BBE 与吉兰-巴雷综合征(GBS)的重叠具有相似的临床和免疫学特征。我们报告了一例继发于 感染的 BBE 与 GBS 重叠病例。该患者出现意识改变、共济失调和眼肌麻痹三联征。检查包括脑脊液细胞蛋白分离、神经传导试验显示 F 波潜伏期延长或消失、脑 MRI 左枕叶高信号和脑电图弥漫性慢波活动。衣原体血清学阳性提示 BBE 综合征为感染后原因。由于意识水平下降伴四肢瘫痪、口咽和呼吸肌无力,他需要人工通气。给予静脉注射免疫球蛋白和甲基强的松龙免疫治疗。他的病情随着治疗得到了很好的恢复。及时识别这种罕见的疾病对于指导治疗很重要。