20例水通道蛋白4抗体阳性的中枢神经系统脱髓鞘疾病特点分析  

Characteristics of Central Nervous System Demyelinating Disorders with Positive Serum AQP4 Antibody in 20 Cases

作　　者：刘波[1] LIU Bo(Department of Neurology,The People's Hospital of Anyang City,Anyang 455000,Henan Province,China)

机构地区：[1]安阳市人民医院神经内科,河南安阳455000

出　　处：《罕少疾病杂志》2024年第9期134-136,共3页Journal of Rare and Uncommon Diseases

摘　　要：目的 探讨水通道蛋白4抗体(AQP4-Ab)阳性的中枢神经系统脱髓鞘疾病患者的临床特点。方法 回顾分析20例住院患者的临床资料,结合临床表现、实验室结果和核磁诊断进行分析。结果 6例符合视神经脊髓炎诊断,13例符合视神经脊髓炎谱系疾病诊断,1例仅有颅内典型病灶。首次发病累及脊髓、视神经和脑干。多数病人合并自身免疫性抗体阳性。结论 AQP4-Ab是视神经脊髓炎、视神经脊髓炎谱系疾病特异性抗体;完善免疫相关指标、脑脊液、脑脊髓MRI检查有助于早期诊断该类疾病;少数AQP4-Ab阳性患者影像学可表现为孤立颅内典型部位病变。Objective To investigate the clinical characteristics of Central Nervous System Demyelinating Disorders with Positive Serum AQP4 Antibody.Methods The clinical data of 20 hospitalized patients were retrospectively analyzed,combined with clinical manifestations,experimental results and NMR diagnosis.Results 6 cases were diagnosed as neuromyelitis optica,13 cases were diagnosed as neuromyelitis optica spectrum disease,and 1 case had only typical intracranial lesions.The first attack affects the spinal cord,optic nerve and brain stem.Most patients are combined with positive autoimmune antibodies.Conclusion AQP4-ab is the specific antibody of neuromyelitis opticum and neuromyelitis opticum spectrum diseases,The improvement of immune related indexes,cerebrospinal fluid and brain spinal cord MRI are helpful to the early diagnosis of this kind of disease,A few AQP4-Ab positive patients may present with isolated intracranial lesions at typical sites on imaging.

关 键 词：水通道蛋白4 视神经脊髓炎 视神经脊髓炎谱系疾病 极后区综合征 

分 类 号：R741[医药卫生—神经病学与精神病学]