后区综合征：AQP4-IgG 阳性 NMOSD 的频率、标准和严重程度。
Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG-positive NMOSD.
机构信息
From the Departments of Neurology (E.S., D.D., M.M., E.F., A.G., B.W., S.J.P.), Laboratory Medicine and Pathology (M.M., E.F., A.G., S.J.P.), and Clinical Research Unit (C.H., J.S.), Mayo Clinic College of Medicine, Rochester, MN; Nuffield Department of Clinical Neurosciences (J.P., M.I.L., S.M.), Oxford; The Walton Centre (A.J., D.W., L.E.), NHS Foundation Trust, Liverpool, UK; Department of Neurology (I.N., K.F., T.T., T.M., Y.T.), Tohoku University Graduate School of Medicine, Sendai; Department of Multiple Sclerosis Therapeutics (K.F.), Fukushima Medical University School of Medicine and Multiple Sclerosis and Neuromyelitis Optica Center, Southern Tohoku Research Institute for NeuroScience, Koriyama, Japan; Department of Neurology (M.L., A.B.), Johns Hopkins University, Baltimore, MD; Departments of Neurology and Neurotherapeutics (B.M.G.), UT Southwestern Medical Center, Dallas, TX; Department of Neurology (T.T.), Yonezawa National Hospital; and Department of Neurology (I.N.), Tohoku Medical and Pharmaceutical University, Sendai, Japan.
出版信息
Neurology. 2018 Oct 23;91(17):e1642-e1651. doi: 10.1212/WNL.0000000000006392. Epub 2018 Sep 26.
OBJECTIVE
To define the frequency, duration, and severity of intractable nausea, vomiting, or hiccups in aquaporin-4-immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and propose diagnostic criteria and a severity scale for area postrema syndrome (APS).
METHODS
An International NMOSD database was interrogated for frequency of APS. Patients with AQP4-IgG-positive NMOSD completed an APS symptom questionnaire. Nausea and vomiting severity was derived from the Pregnancy-Unique Quantification of Emesis and Nausea (PUQE) score. The diagnostic criteria, severity scale, and immunotherapy response was applied to a prospective validation cohort of patients from multiple centers.
RESULTS
Analysis of an international database for AQP4-IgG-seropositive NMOSD (n = 430) revealed a high prevalence of isolated APS attacks (onset 7.1%-10.3%; subsequent 9.4%-14.5%) across continents. For 100 patients with 157 episodes of APS, nausea (n = 127, 81%) lasted for a median of 14 days (range 2-365), vomiting (113, 72%) with a median of 5 episodes/d (2-40) lasted 1-20 minutes, and hiccups (102, 65%) lasted a median of 14 days (2-365). Symptoms consistently and completely resolved following immunotherapy. Data were used to propose APS diagnostic criteria and repurpose PUQE score (hiccups severity grade based on symptom duration). The clinical utility was demonstrated in a prospective validation cohort.
CONCLUSION
Isolated APS attacks are frequently encountered both at onset and during the NMOSD course. The diagnostic criteria proposed here will assist clinicians in recognizing APS. Diagnosis of an APS attack earlier than 48 hours is possible if a dorsal medulla lesion is detected. Accurate diagnosis and evaluation of APS attack severity will assist in outcome measurement in NMOSD clinical trials.
目的
确定水通道蛋白 4 免疫球蛋白 G（AQP4-IgG）阳性视神经脊髓炎谱系疾病（NMOSD）患者难治性恶心、呕吐或呃逆的频率、持续时间和严重程度，并提出前庭神经下核综合征（APS）的诊断标准和严重程度分级。
方法
通过 NMOSD 国际数据库查询 APS 的发生频率。AQP4-IgG 阳性 NMOSD 患者填写 APS 症状问卷。恶心和呕吐的严重程度来自妊娠特异性呕吐和恶心量表（PUQE）评分。将诊断标准、严重程度分级和免疫治疗反应应用于多中心前瞻性验证队列的患者。
结果
对国际 AQP4-IgG 阳性 NMOSD 数据库（n = 430）的分析显示，在整个大陆，孤立性 APS 发作的患病率较高（首次发作 7.1%-10.3%；随后发作 9.4%-14.5%）。在 100 例 157 次 APS 发作的患者中，恶心（n = 127，81%）持续时间中位数为 14 天（范围 2-365 天），呕吐（n = 113，72%）的中位数为 5 次/天（2-40 次），持续 1-20 分钟，呃逆（n = 102，65%）持续时间中位数为 14 天（2-365 天）。免疫治疗后症状均完全缓解。这些数据用于提出 APS 诊断标准，并重新利用 PUQE 评分（根据症状持续时间的呃逆严重程度分级）。在一项前瞻性验证队列中验证了该标准的临床实用性。
结论
孤立性 APS 发作在 NMOSD 发病和病程中均较常见。这里提出的诊断标准将有助于临床医生识别 APS。如果检测到背侧髓质病变，则有可能在 48 小时内更早诊断 APS 发作。准确诊断和评估 APS 发作严重程度将有助于 NMOSD 临床试验中的结局测量。
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