摘要
目的描述因视神经脊髓炎谱系疾病(NMOSD)累及脑干而出现急性呼吸衰竭(ARF)患者的临床特征,提出“NMOSD脑干危象”的概念,以提高临床医生对此型患者的认识以及提升对发生ARF患者的预警。方法报道NMOSD累及脑干并出现ARF 2例患者临床资料,结合国内外文献复习,对该类患者的临床特点进行分析和总结。结果病例1,17岁女性,临床表现为顽固性恶心、呕吐、面瘫、伸舌无力、四肢无力,因ARF而行气管插管,血清AQP4抗体阳性,经免疫治疗及对症治疗后拔除气管插管,目前生活完全自理。病例2,35岁女性,临床表现为持续性恶心、呃逆、吞咽困难、四肢无力,因ARF气管插管,血清AQP4抗体阳性,免疫治疗后症状好转。结论NMOSD病灶累及延髓背侧的呼吸中枢是出现ARF的重要原因。急性吞咽困难、伸舌无力、四肢无力对出现ARF有一定的警示作用,患者症状可突然加重并危及生命。为了引起临床医生的重视,建议将上述合并呼吸衰竭的症候群称为“NMOSD脑干危象”。未接受规范治疗、合并畸胎瘤及青少年起病可能是出现NMOSD脑干危象的危险因素。
Aim To describe the clinical features of patients with acute respiratory failure(ARF)due to brainstem involvement in neuromyelitis optica spectrum disorder(NMOSD),and propose the concept of brainstem crisis in order to improve the awareness of neurologists and the early warning of acute respiratory failure in this type of patients.Methods Two cases of NMOSD involving brainstem and acute respiratory failure were reported.Combined with domestic and foreign literature,the clinical characteristics of these patients were analyzed and summarized.Results Case 1 was a 17-year-old female with clinical manifestations of intractable nausea,vomiting,facial paralysis,tongue extension weakness,and limb weakness.Due to respiratory failure,tracheal intubation was performed and serum AQP4 antibody was positive.The tracheal intubation was removed after immunotherapy and symptomatic treatment,and she now lives completely by herself.Case 2,a 35-year-old female,presented with persistent nausea,hiccups,dysphagia,and limb weakness.She was tracheal intubated due to respiratory failure.Serum AQP4 antibody was positive,and her symptoms improved after immunotherapy.Conclusion Lesions involving the respiratory center of the dorsal medulla are an important cause of acute respiratory failure in NMOSD patients.Acute dysphagia,tongue weakness,and limb weakness are early warning signs of acute respiratory failure,which can actually threaten the life of the patients.In order to attract the attention of clinicians,it is recommended to call the syndrome combined with respiratory failure brainstem crisis of NMOSD.The risk factors of brainstem crisis in NMOSD patients may be non-treatment,teratoma and adolescent onset.
作者
李瑞球
邓波
杨文波
刘小妮
李亚蓉
金晶
耿艳芹
张祥
陈向军
俞海
LI Rui-qiu;DENG Bo;YANG Wen-bo;LIU Xiao-ni;LI Ya-rong;JIN Jing;GENG Yan-qin;ZHANG Xiang;CHEN Xiang-jun;YU Hai(Department of Neurology,Huashan Hospital,Fudan University,Shanghai 200040,China;Institute of Neurology,Fudan University,Shanghai 200040,China;National Center for Neurological Disorders,Shanghai 200040,China;Jiangnan University Medical Center,Wuxi 214002,China;School of Biomedical Engineering,Shanghai Jiaotong University,Shanghai 200031,China)
出处
《中国临床神经科学》
2025年第2期193-199,共7页
Chinese Journal of Clinical Neurosciences
基金
国家卫健委,2020年医疗服务与保障能力提升(医疗卫生能力建设)项目
国家和省级重大疾病多学科合作诊疗能力建设项目:基于队列的神经系统自身免疫与感染疾病多学科精准诊治平台建设。
