| 刘建盛,庄美婷,李格飞,王 萍,乔 园,夏登云,陈金梅.以急性脑干综合征为首发表现的NMOSD的临床和MRI诊断分析[J].,2020,(1):68-71 |
| 以急性脑干综合征为首发表现的NMOSD的临床和MRI诊断分析 |
| Clinical Manifestations and MRI Characteristics of Neuromyelitis Optica Spectrum Disorders Initiated with Acute Brainstem Syndrome |
| 投稿时间：2019-05-31 修订日期：2019-06-28 |
| DOI：10.13241/j.cnki.pmb.2020.01.014 |
| 中文关键词: 视神经脊髓炎谱系疾病 急性脑干综合征 脑脊液 水通道蛋白4 磁共振 诊断 |
| 英文关键词: Neuromyelitis optica spectrum disorder Acute brainstem syndrome Cerebrospinal fluid Aquaporin-4 Magnetic resonance imaging Diagnosis |
| 基金项目:国家自然科学基金项目(81701267)；上海交通大学医工(理)交叉基金项目(YG2016QN01) |
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| 中文摘要: |
| 摘要 目的：探讨以急性脑干综合征(ABS)为首发表现的视神经脊髓炎谱系疾病(NMOSD)的临床和MRI表现，以提高对该病的诊断水平。方法：回顾性分析17例首发表现为ABS的NMOSD患者临床资料，包括脑脊液常规、生化及寡克隆区带，血清水通道蛋白4抗体(AQP4- IgG)，头颅与脊髓MRI表现，并分析其特点。结果：共纳入男性3例，女性14例，发病年龄20~43岁，平均发病年龄33.5岁，88.2%患者以恶心、呕吐、顽固性呃逆等胃肠症状就诊，发作病程7天~47周，平均8周。脑脊液检查多呈轻中度炎性反应，2例白细胞计数＞50×106/L。脑脊液蛋白平均0.32 g/L(0.15~1.17 g/L)，OBs检测阳性率为11.8%，血清AQP4-IgG阳性率为76.5%。64.7%病例早期MRI表现延髓背侧中央导水管周围异常信号，无明显强化；脊髓未见受累。结论：中青年女性以ABS为首发症状时应警惕NMOSD的可能，脑脊液检查、血清AQP4抗体阳性以及MRI表现具有一定的特征性，有助于早期诊断。 |
| 英文摘要: |
| ABSTRACT Objective: To increase the awareness on neuromyelitis optica spectrum disorders (NMOSD) initiated with acute brainstem syndrome (ABS) by presenting clinical manifestations and magnetic resonance imaging (MRI) findings on this disease. Methods: The characteristics of seventeen patients with ABS as initial presentation were retrospectively analyzed, including the cerebrospinal fluid (CSF), serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) and neuroimaging. Results: A total of three male and fourteen female patients were included, with a median age of 33.5 years (range, 31~63 years). The initial evaluation in 88.2% was gastroenterologic. Vomiting lasted a median of 8 weeks (range, 7 days-47 weeks). Most patients had a slightly higher number of white blood cells in CSF, and pleocytosis＞50×106 leukocytes was seen in 2 cases. The mean of protein concentrations in CSF was 0.32 g/L (range, 0.15~1.17 g/L). Only 11.8% were positive for oligoclonal bands in CSF, but a higher percentage (76.5%, 13/17) of patients was seropositive for AQP4-IgG. In examination of MRI, the 64.7% of ABS patients had medulla-predominant involvements in the sagittal view and dorsal-predominant involvements in the axial view. None of the spinal cord lesions were detected when the initial episode of ABS. Conclusion: ABS should be considered as an inaugural form of NMOSD, especially in young and middle-aged female patients. Laboratory CSF criteria, positive serum AQP4-IgG and MRI features are used to support an early and accurate diagnosis of NMOSD. |
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