摘要
目的提高对视神经脊髓炎谱系疾病（NMOSD）的认识。方法报道1例以脑干症状为首发、继以急性横贯性脊髓炎的NMOSD患者,结合文献复习进行分析。结果青年女性患者,发病时表现为左侧味觉减退以及左侧面部瘙痒,继以出现急性横贯性脊髓炎。MRI显示延髓右侧和左侧桥脑被盖部、中脑导水管周围长T1、长T2信号病灶,脊髓MRI显示颈1~5长T2信号病灶;血清水通道蛋白4（AQP4）抗体阳性。患者符合AQP4抗体阳性NMOSD的诊断标准。结论 NMOSD常有脑部症状,尤其是脑干症状。部分患者可以脑干症状为首发临床表现,对此类患者应及早行AQP4抗体检测,以明确诊断及早干预。
Aim To improve the recognition of neuromyelitis optica spectrum disorders（NMOSD）.Methods A case of NMOSD initiated with brainstem symptoms,followed by acute transverse myelitis,was reported,and related literature was reviewed.Results A young female initiated with hypogeusia on the entire left side of the tongue and pruritus on the left face,and then developed acute transverse myelitis.Brain MRI revealed hypointense lesions on T1 weighted image and hyperintense lesions on T2 weighted image in the right side of medulla oblongata,left midpontine tegmentum,and periaqueduct.Spinal cord MRI showed a longitudinally extensive lesion involving C1 to C5.AQP4 antibodies were positive in serum.The patient fulfilled with the diagnostic criteria of NMOSD with positive AQP4 antibodies.Conclusion NMOSD often present with brain symptoms,especially brainstem symptoms.Some patients may initiate with different brainstem symptoms.Early detection of AQP4 antibodies is crucial for diagnosis.
出处
《中国临床神经科学》
2016年第3期324-327,347,共5页
Chinese Journal of Clinical Neurosciences
