■探讨最初表现为急性脑干症状的视神经脊髓炎谱系障碍(NMOSD)患者的临床特征和预后。
■收集两个医疗中心收治的NMOSD患者的临床资料。临床特点,实验室数据,分析以急性脑干表现为首发症状(NMOSD-BSMIS)的NMOSD患者的神经影像学特征和预后。比较NMOSD-BSMIS患者和以其他表现为首发症状的NMOSD患者(NMOSD-OMIS)的临床特征和预后。
■52名患者(18.37%,52/283)最初表现为急性脑干症状。顽固性恶心,呕吐或打嗝,复视,眩晕,头痛,面部感觉减退是大多数患者的最初症状。血清水通道蛋白4(AQP4)-IgG抗体阳性的患者比例为81.63%(40/49)。MRI显示病灶通常位于病灶后,延髓背侧,桥和第四脑室周围的其他区域。早期误诊率为46.15%。与未误诊组相比,NMOSD-BSMIS组患者发病年龄较大,在误诊组中,神经内科作为第一科室的患者比例较低。NMOSD-BSMIS患者年复发率明显高于NMOSD-OMIS患者(P<0.01)。
■NMOSD患者最初可能会出现不同的脑干症状。NMOSD-BSMIS早期误诊率高。此外,如果患者年龄较大或最初进入非神经科,他们更容易被误诊。此外,早期NMOSD-BSMIS的年复发率较大。
UNASSIGNED: To explore the clinical features and prognosis of patients with neuromyelitis optica spectrum disorder (NMOSD) initially presenting with acute brainstem symptoms.
UNASSIGNED: The clinical data of NMOSD patients admitted to two medical centers were collected. The clinical characteristics, laboratory data, neuroimaging features and prognoses of patients with NMOSD with acute brainstem manifestations as initial symptoms (NMOSD-BSMIS) were analyzed. The clinical features and prognosis of patients with NMOSD-BSMIS and patients with NMOSD with other manifestations as initial symptoms (NMOSD-OMIS) were compared.
UNASSIGNED: Fifty-two patients (18.37 %, 52/283) initially presented with acute brainstem symptoms. Intractable nausea, vomiting or hiccups, diplopia, vertigo, headache, and facial hypoesthesia were the initial symptoms in most of the patients. The percentage of patients who were positive for serum aquaporin 4 (AQP4)-IgG antibodies was 81.63 % (40/49). MRI revealed that the lesions were usually located in the postrema, dorsal medulla oblongata, pons and other areas around the fourth ventricle. The early-stage misdiagnosis rate was 46.15 %. Compared with those in the non-misdiagnosed group, the age of onset of patients in the NMOSD-BSMIS group was older, and the proportion of patients admitted to the neurology department as the first department was lower in the misdiagnosed group. The annual relapse rate of patients who underwent NMOSD-BSMIS was significantly greater than that of patients who underwent NMOSD-OMIS (P < 0.01).
UNASSIGNED: NMOSD patients can initially present with different brainstem symptoms. The early misdiagnosis rate of NMOSD-BSMIS is high. Moreover, if patients are older or initially admitted to nonneurological departments, they are more likely to be misdiagnosed. Moreover, the annual recurrence rate of NMOSD-BSMIS is greater in the early stage.