视神经脊髓炎谱系疾病诱发体位性低血压的临床特征分析并文献复习

Clinical analysis of orthostatic hypotension in neuromyelitis optica spectrum disorder and literature review

目的探讨视神经脊髓炎谱系疾病(NMOSD)诱发体位性低血压患者的临床表现及头颅、脊髓磁共振成像(MRI)特点并进行相关文献复习。方法回顾性收集2012年2月至2022年6月首都医科大学附属北京友谊医院神经内科收治的51例NMOSD患者的临床资料。记录患者的性别、发病或复发年龄、既往病史、临床表现、血压及心率变化、相关实验室检查及MRI检查结果等。对其中8例临床上诱发严重的体位性低血压患者进行临床实验室特征及头颅、脊髓MRI分析,同时与未发生体位性低血压的患者进行相关统计分析。结果51例NMOSD患者中8例合并严重的体位性低血压,临床表现为其所致的反复的晕厥、头晕、心慌、大汗及乏力等。8例患者均大于60岁,中位数为65岁,其中6例血清AQP4为阳性,男女比例为3∶1;其中1例为首发症状,且出现在顽固性恶心、呕吐之后,MRI特点为极后区脱髓鞘性病变;7例为病程中出现,MRI检查特点为极后区或颈髓髓内脱髓鞘性病变表现。免疫治疗后有3例完全缓解,5例部分缓解。与未发生体位性低血压的患者相比,NMOSD中发生体位性低血压患者的男性比率、合并心血管系统疾病率、合并脊髓型颈椎病率、颈髓髓内病变发生率较高,血压失调不稳发生率较低,差异均有统计学意义(P<0.05)。结论体位性低血压所致的临床症状应在视神经脊髓炎谱系疾病患者中应引起重视,尤其是老年男性合并慢性疾病或脊髓型颈椎病患者,同时需及时检查颈椎MRI明确有无髓内病变,严密监测血压变化。

Objective To explore the clinical and MRI manifestations with orthostatic hypotension caused by neuromyelitis optica spectrum diorders and review relevant literature.Methods The clinical data of 51 NMOSD patients admitted to the Department of Neurology,Beijing Friendship Hospital Affiliated to Capital Medical University from February 2012 to June 2022 were retrospectively collected.Their genders,age of onset or relapse,past medical history,clinical manifestations,blood pressure and heart rate,laboratory examination and imaging materials were recorded.Their clinical profiles and MRI features were analyzed in 8 patients with orthostatic hypotension.Results Among 51 NMOSD patients,8 patients were complicated with severe orthostatic hypotension.The clinical manifestations were recurrent syncope,dizziness,palpitation,hyperhidrosis and fatigue.All the 8 patients were beyond 60 years old.The AQP4 of serum were positive in 6 patients,the ratio of male to female was 3∶1.Among them,1 case was the first symptom and appeared after intractable nausea and vomiting.The MRI features were demyelinating lesions in the posterior polar region.7 cases occurred during the course of the disease,and the MRI features were demyelinating lesions in the posterior polar region or cervical spinal cord.3 patients got complete remission after immunosuppressive therapy,but 5 patients achieved partial remission.Compared with patients without orthostatic hypotension,the incidence of male ratio,cardiovascular disease,cervical spondylotic myelopathy and cervical intramedullary lesions of patients with orthostatic hypotension in NMOSD were higher,and the incidence of blood pressure instability was lower,and the differences were statistically significant(P<0.05).Conclusion Orthostatic hypotension should be evaluated carefully in patients with NMOSD,particularly the male patients with chronic disease rate,cervical spondylotic myelopathy rate.These patients need the examination of cervical MRI timely and careful monitor of blood pressure.