摘要
目的分析以脑干受损症状为首发表现的视神经脊髓炎谱系疾病(NMOSD)患者的临床特征及预后情况,以提高临床工作者对此病的认识,减少早期误诊率。方法收集2009年1月~2020年8月本院收治的符合国际NMOSD诊断标准的185例患者的临床资料,分析其中的28例以脑干受损症状为首发表现的NMOSD患者的临床特点、体液学检查、影像学特征及预后。比较以脑干受损症状为首发表现的NMOSD与以其他症状为首发表现的NMOSD的临床特点及预后。结果以脑干受损症状为首发表现患者共28例(15.14%,28/185),其中17例患者以顽固性恶心、呕吐和/或呃逆为主要症状,4例以复视为主要症状,其余以头晕、面部麻木、瘙痒等为主要症状。血清水通道蛋白4(AQP4)-IgG抗体阳性率为(76%,19/25),MRI检查显示病灶通常位于最后区、延髓背侧、脑桥和第四脑室周围区域。28例患者中,15例被误诊,误诊患者较未误诊患者,神经内科作为首诊科室的比例低(P<0.05)。与其他症状起病的NMOSD患者相比,以脑干受损症状为首发表现的NMOSD患者的误诊率和年复发率均更高(均P<0.01)。结论以脑干受损症状为首发表现的NMOSD患者在临床上并不少见,病损通常位于最后区、延髓背侧、脑桥和第四脑室周围区域。血清AQP4抗体、头颅MRI检查有助于诊断。此类患者早期误诊率高,首诊于非神经科科室时更易被误诊,且较其他症状起病的NMOSD更易复发。
Objective To explore the clinical features and prognosis of patients with neuromyelitis optica spectrum disorder initially presenting with acute brainstem symptoms.Methods The clinical data of 185 patients with neuromyelitis optica spectrum disease who were admitted to the Affiliated Hospital of Xuzhou Medical University from January 1,2009 to August 1,2020 and met the international diagnostic criteria for NMOSD were collected.The clinical features,laboratory examinations,neuroimaging characters and prognosis of 28 patients with NMOSD initially presenting with acute brainstem symptoms were analyzed.The clinical features and prognosis of patients with NMOSD with acute brainstem symptoms as initial presentation were compared with patients with NMOSD with other symptoms.Results There were 28 patients(15.14%,28/185)initially presenting with acute brainstem symptoms.Intractable nausea,vomiting and/or hiccup were the main symptoms in 17 patients,diplopia was the main symptom in 4 patients,and dizziness,facial numbness,pruritus,etc.were the main symptoms in others.The positive rate of serum aquaporin 4(AQP4)-igG antibodies was 76%(19/25).MRI revealed that lesions were usually located in the area postrema,dorsal medulla oblongata,pons and area around the fourth ventricle.Among the 28 patients,15 patients were misdiagnosed.Compared with the non-misdiagnosed patients group,the ratio of neurology department as first visit department was lower in the misdiagnosed patients group(P<0.05).The misdiagnosis rate and annual recurrence rate of 28 patients were significantly higher than the others(both P<0.01).Conclusion NMOSD patients with brain stem injury as the first manifestation are not uncommon clinically,and the lesions are usually located in the final region,the dorsal medulla oblongata,the pons,and the area around the fourth ventricle.Serum AQP4 antibody and head MRI are helpful for diagnosis.Such patients have a high rate of early misdiagnosis,and are more likely to be misdiagnosed when first diagnosed in non-neurologic departments,and more likely to relapse than NMOSD with other symptoms.
作者
李震
吴晗
罗聪
祖洁
张伟
徐传英
崔桂云
LI Zhen;WU Han;LUO Cong;ZU Jie;ZHANG Wei;XU Chuanying;CUI Guiyun(Department of Neurology,The Affiliated Hospital of Xuzhou Medical University,Xuzhou 221002,Jiangsu,China)
出处
《西部医学》
2023年第5期759-764,共6页
Medical Journal of West China
作者简介
通讯作者:崔桂云,E-mail:teachercuigy@126.com。
