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

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>50×10^6/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。

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-Ig G) 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>50×10^6 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-Ig G and MRI features are used to support an early and accurate diagnosis of NMOSD.