背景:后面积综合征(APS)被认为是视神经脊髓炎(NMO)诊断的核心特征。孤立性APS可在NMO发病时发生,并经常导致诊断延迟。伴随着毁灭性的继发性神经缺陷。迄今为止,很少有研究描述APS发作性视神经脊髓炎谱系障碍(APSO-NMOSD)。
目的:我们的目的是描述APSO-NMOSD患者的临床和影像学特征,这些患者最初被误诊为郑州患者,中国。
方法:我们确定了15例患者以APS为首发表现,根据2015年NMOSD国际共识诊断标准,并回顾了他们的人口统计,临床,实验室,和磁共振成像(MRI)数据。
结果:15名患者(3名男性,我们的研究包括12名年龄在14-50岁之间的女性)。所有患者出现APS,包括顽固性恶心,呕吐,或打嗝(INVH)作为最初的表现;许多经历了诊断延迟。11例患者血清AQP4阳性,1例患者髓鞘少突胶质细胞糖蛋白(MOG)阳性。所有患者在矢状MRI上均有线性髓质病变或线性髓质脊髓病变。轴性延髓图像上的“倒V形”,代表一个涉及后区域的病变,在这项研究中,有7名患者被发现。
结论:APS可以作为NMOSD的唯一和初始表现而发生,常导致疾病早期误诊。在MRI检查中识别具有“倒V”征和线性髓质病变的患者可以帮助快速识别APS患者并避免进一步的诊断延迟。
BACKGROUND: Area postrema syndrome (APS) is recognized as a core feature in neuromyelitis optica (NMO) diagnosis. Isolated APS can occur at NMO onset and frequently results in a delay of diagnosis, along with devastating secondary neurologic deficits. To date, few studies have characterized APS-onset neuromyelitis optica spectrum disorder (APSO-NMOSD).
OBJECTIVE: We aimed to describe the clinical and radiologic features of patients with APSO-NMOSD who are initially misdiagnosed in a cohort of patients from Zhengzhou, China.
METHODS: We identified 15 patients who presented with APS as an initial manifestation, based on the 2015 international consensus diagnostic criteria for NMOSD, and reviewed their demographic, clinical, laboratory, and magnetic resonance imaging (MRI) data.
RESULTS: Fifteen patients (3 men, 12 women) aged 14-50 years old were included in our
study. All patients presented with APS that included intractable nausea, vomiting, or hiccups (INVH) as the initial manifestation; many experienced a delay in diagnosis. Serum
AQP4 was positive in eleven patients and myelin oligodendrocyte glycoprotein (MOG) in one patient. All patients had a linear medullary lesion or a linear medulla-spinal lesion on sagittal MRI. An \"inverted V sign\" on axial medulla oblongata images, representing a lesion involving the area postrema, was noted in seven patients in this
study.
CONCLUSIONS: APS can occur as a sole and initial manifestation of NMOSD, often leading to misdiagnosis in the early process of disease. Identifying patients with an \"inverted V\" sign and a linear medullary lesion upon MRI examination can help to quickly identify APS patients and avoid further diagnostic delays.