以顽固性恶心呕吐、呃逆为表现的极后区综合征在NMOSD中患者的临床特征及误诊分析

Analysis of the clinical characteristics and misdiagnosis of area postrema syndrome manifesting as intractable nausea,vomiting,and hiccups in neuromyelitis optica spectrum disorders

目的研究以顽固性呕吐、呃逆为主要表现的极后区综合征在视神经脊髓炎谱系疾病(NMOSD)中患者的临床特征及误诊情况,为临床医生提供参考,减少漏误诊。方法病例系列研究。回顾性分析2019年1月至2021年7月就诊于解放军总医院第一医学中心神经内科医学部的NMOSD患者资料,对出现极后区综合征(APS)的患者表现及误诊误治情况进行分析。采用SPSS25.0软件进行统计学分析。结果共纳入NMOSD患者207例,其中男性21例、女性186例,发病年龄5~72(39±15)岁。血清水通道蛋白4抗体阳性率82.6%(171/207)。35.7%(74/207)的NMOSD患者病程中出现APS,其中70.3%(52/74)以此为疾病的首发症状,29.7%(22/74)为非首发症状,两者的误诊率分别为90.4%(47/52)和50.0%(11/22)。作为首发症状,APS期间19.2%(10/52)的患者仅表现为APS,80.8%(42/52)的患者同时伴随其他神经科症状。出现APS,最常首诊科室为消化内科和综合内科,分别占到54.1%和17.6%,最常见被误诊为消化系统疾病,平均误诊中位数时长37 d。结论APS是NMOSD的常见症状,误诊率高;APS期间常出现其他伴随症状;提高对该病及该综合征的认识,详细的病史和查体,对于减少和避免误诊至关重要。

Objective To investigate the misdiagnosis of area postrema syndrome(APS)manifesting as intractable nausea,vomiting and hiccups in neuromyelitis optic spectrum disease(NMOSD)and reduce the risk of misdiagnosis.Methods We retrospectively analyzed data from NMOSD patients attending the Department of Neurology at the First Medical Center of PLA General Hospital between January 2019 and July 2021.SPSS25.0 was then used to analyze the manifestations,misdiagnosis,and mistreatment of APS.Results A total of 207 patients with NMOSD were included,including 21 males and 186 females.The mean age of onset was 39±15 years(range:5-72 years).The proportion of patients who were positive for serum aquaporin 4 antibody was 82.6%(171/207).In total,35.7%(74/207)of the NMOSD patients experienced APS during the disease course;of these patients,70.3%(52/74)had APS as the first symptom and 29.7%(22/74)had APS as a secondary symptom.The misdiagnosis rates for these conditions were 90.4%(47/52)and 50.0%(11/22),respectively.As the first symptom,19.2%(10/52)of patients during APS presented only with intractable nausea,vomiting and hiccups;80.8%(42/52)of patients experienced other neurological symptoms.The Departments of Gastroenterology and General Medicine were the departments that most frequently made the first diagnosis of APS,accounting for 54.1%and 17.6%of patients,respectively.The most common misdiagnoses related to diseases of the digestive system and the median duration of misdiagnosis was 37 days.Conclusions APS is a common symptom of NMOSD and is associated with a high rate of misdiagnosis.Other concomitant symptoms often occur with APS.Gaining an increased awareness of this disease/syndrome,obtaining a detailed patient history,and performing physical examinations are essential if we are to reduce and avoid misdiagnosis.