成人复发型急性播散性脑脊髓炎的诊断学特征并文献复习

Diagnostic features of recurrent acute disseminated encephalomyelitis in adults and literature review

目的探讨成人复发型急性播散性脑脊髓炎的诊断学特征。方法回顾性分析泰安市立医院神经内科收治的1例成人复发型急性播散性脑脊髓炎患者2018年5月至2020年5月3次发病的临床资料,总结急性播散性脑脊髓炎的诊断学特征,并复习相关文献。结果患者3次发病前均有感染病史,每次发病均有头晕、言语不清、步态不稳。查血、脑脊液寡克隆带(-);血、脑脊液水通道蛋白4(AQP4)抗体(-),髓鞘碱性蛋白(MBP)抗体(-),髓鞘少突胶质细胞糖蛋白(MOG)抗体(-),神经节苷脂谱抗体(-)。头颅磁共振成像(MRI)平扫提示不除外脱髓鞘改变,增强未见明显强化;MRI动脉自旋标记(ASL)灌注成像提示脑内部分呈低灌注表现,考虑脱髓鞘病变;磁共振波谱成像(MRS)符合脱髓鞘病变MRS表现。3次发病激素冲击治疗后症状均好转。结论急性播散性脑脊髓炎的临床表现复杂多样,需借助血清抗体、影像学及神经电生理方能正确诊断。

ObjectiveTo explore the diagnostic features of recurrent acute disseminated encephalomyelitis(ADEM)in adults.MethodsThe clinical data of an adult patient with recurrent ADEM in Neurology Department of Taian Municipal Hospital from May 2018 to May 2020 were retrospectively analyzed.The diagnostic features of ADEM were summarized and relevant literatures were reviewed.ResultsThe patient had a history of infection before the onset of 3 episodes,and had dizziness,slurred speech,and unsteady walking in each episode.Blood and cerebrospinal fluid oligoclonal zone(-).Blood and cerebrospinal fluid aquaporin 4(AQP4)antibody(-),myelin basic protein(MBP)antibody(-),myelin oligodendrocyte glycoprotein(MOG)antibody(-)and ganglioside profile antibody(-).MRI of the brain did not exclude changes in demyelination.There was no obvious enhancement in brain MRI enhancement.MRI arterial spin labeling(ASL)perfusion imaging suggested that the brain was hypoperfused,and demyelinating disease was considered.Magnetic resonance spectroscopy(MRS)conformed to the MRS manifestations of demyelinating lesions.Symptoms improved after hormone shock therapy for 3 episodes.ConclusionThe clinical manifestations of ADEM are complex and varied,which can be correctly diagnosed by means of serum antibody,imaging and neuroelectrophysiology.