极后区综合征在水通道蛋白-4-免疫球蛋白G阳性视神经脊髓炎谱系疾病中的多中心临床分析

Multicenter clinical analysis of area postrema syndrome in aquaporin-4-immunoglobulin G-positive neuromyelitis optica spectrum disorder

目的分析极后区综合征(APS)在水通道蛋白-4-免疫球蛋白G(AQP4-IgG)阳性视神经脊髓炎谱系疾病(NMOSD)的临床表型、影像特征,提出可行的诊断标准及严重程度的量化标准。方法通过回顾性病例研究从131例AQP4-IgG阳性的NMOSD患者中筛选的21例存在APS的患者的临床资料及头颈和脊髓的影像资料等,分析APS临床表型,同时使用改良的孕妇专用呕吐和恶心量表(PUQE)量化评估患者在治疗前、治疗7 d后的严重程度。结果AQP4-IgG阳性NMOSD患者首发症状存在APS患者中女性多见(17/21),发病中位年龄(37.6±16.8)岁。其中以孤立性极后区综合征(IAPS)为首发症状患者12例(9.16%),APS合并视神经炎或脊髓炎患者9例(6.87%)。所有患者均在发病后的3 d内行头颅及脊髓磁共振成像,13例(61.90%)显示延髓背内侧区域T2/液体衰减反转恢复序列高信号改变,其中7例存在延髓—脊髓延续性病变,4例为孤立性延髓病变,2例延髓病变合并视神经强化。12例患者(57.14%)存在误诊,均误诊为消化系统疾病。21例患者确诊后接受了免疫治疗(静脉注射甲基强的松龙(16例)、静脉丙种球蛋白(4例)、血浆置换(1例))。治疗前通过改良的PUQE对顽固性呃逆和(或)恶心、呕吐(INH)症状评级,其中16例(76.19%)严重,4例(19.05%)中度,1例(4.76%)轻度。治疗7 d后改良的PUQE评估显示17例患者(80.95%)症状完全消失。结论无其他明显诱因的发作性或持续性的急性或亚急性INH,症状持续≥48 h且对症治疗或非免疫治疗无明显缓解时,建议及时行血清AQP4-IgG的检测以明确诊断,影像学检查是APS诊断的支持性依据。

Objective To explore the clinical and neuroimaging characteristics of aquaporin-4-immunoglobulin G(AQP4-IgG)-positive neuromyelitis optica spectrum disorder(NMOSD)with the area postrema syn-drome(APS).Methods 21 cases out of 131 NMOSD patients with AQP4-IgG-positive diagnosed were screened for APS.All the clinical data and imaging profiles of the head,neck,and spinal cord were collected to analyze the phenotypes of the APS.Modified pregnancy-unique quantification of emesis and nausea(PUQE)was used to quantitatively evaluate the severity of APS before treatment and 7 days after treatment.Results In the 21 cases of AQP4-IgG-positive NMOSD with APS as the initial symptom,most were female(17max 21),and their median age of onset was(37.6±16.8)years old.Among them,isolated area postrema syndrome(IAPS)was the initial symptom in 12 cases(9.16%),APS with optic neuritis or myelitis in 9 cases(6.87%).Magnetic resonance imaging of the head and spinal cord was performed in all patients within 3 days after onset.13 cases(61.90%)showed high signal changes of T2/fluid attenuated inversion recovery sequence involving the dorsomedial medulla oblongata.Among them,7 cases had medulla oblongataspinal cord continuous lesions,4 cases were isolated medulla oblongata lesions and 2 cases were medulla oblongata complicated with optic nerve enhancement lesions.Twelve patients(57.14%)were misdiagnosed as digestive system diseases.After diagnosis,21 cases received immunotherapy intravenous methylprednisolone(16 cases),intravenous gamma globulin(4 cases)and plasma exchange(1 case).Before treatment,intractable nausea or vomiting and hiccups(INH)were rated by the improved PUQE criteria,with 16 cases(76.19%)being severe,4(19.05%)moderate and 1(4.76%)mild.After 7 days of treatment,the improved PUQE standard evaluation showed that the symptoms of 17 patients(80.95%)disappeared completely.Conclusion Unexplained INH lasted for 48 hours or above,especially z lack of response to symptomatic therapies;thus unimmunotherapy and serum AQP4-IgG needed to be performed in the suspected patients.Neuroimaging examination is the supporting basis for the diagnosis of APS.