Department of Neurology, the First Affiliated Hospital of Fujian Medical University, Fuzhou, Fujian, 350005, China.
Fujian Key Laboratory of Molecular Neurology, Institute of Neuroscience, Fujian Medical University, Fuzhou, Fujian, 350004, China.
BMC Neurol. 2024 May 21;24(1):165. doi: 10.1186/s12883-024-03667-3.
Neuromyelitis Optica Spectrum Disorder (NMOSD) is an inflammatory autoimmune disease with high risk of recurrence and disability, the treatment goal is a recurrence free state. Area postrema (AP) is one of the most common involved area of NMOSD, which may have a particular significance in the pathogenesis of NMOSD and clinical heterogeneity. Our study is to investigate the clinical and recurrent characteristics AP onset NMOSD patients.
A retrospective study was done in a cohort of 166 AQP4-IgG seropositive NMOSD patients which were identified by the 2015 IPND criteria. The patients were divided into AP onset (APO-NMOSD) group and non-AP onset (NAPO-NMOSD) group based on the initial episode location. Clinical features and recurrence differences of two groups were compared.
The APO-NMOSD group and NAPO-NMOSD group had a population ratio of 24:142. APO-NMOSD patients were younger (34.6y VS 42.3y, P = 0.013), had lower EDSS at first episode (0.7 VS 4.2, p = 0.028) and last follow up (1.9 VS 3.3, p = 0.001), more likely to have multi-core lesions at the first attack (33.3% VS 9.2%, P = 0.001). Also, they had a higher annual recurrence rate (0.4 ± 0.28 VS 0.19 ± 0.25, P = 0.012). In natural course NMOSD patients without immunotherapy, APO-NMSOD had a shorter time of first relapse (P < 0.001) and higher annual recurrence rate (0.31 ± 0.22 VS 0.16 ± 0.26, P = 0.038) than NAPO-NMOSD. APO-NMOSD group also have a higher risk of having the first relapsing compared to optic neuritis onset-NMOSD (HR 2.641, 95% CI 1.427-4.887, p = 0.002) and myelitis onset-NMOSD group (HR 3.593, 95% CI 1.736-7.438, p = 0.001). Compared to NAPO-NMOSD, APO-NMOSD has a higher likelihood of brainstem recurrence (28.6% vs. 4.7%, p<0.001) during the first recurrence, while NAPO-NMOSD is more susceptible to optic nerve involvement (10.7% vs. 41.1%, p = 0.01).
AQP4-IgG seropositive NMOSD patients with AP onset are youngers and have higher risk of recurrence. Clinicians should pay attention to AP damage in NMOSD, as it indicates a potential risk of recurrence.
Retrospectively registered.
视神经脊髓炎谱系疾病（NMOSD）是一种具有高复发和致残风险的炎症性自身免疫性疾病，其治疗目标是实现无复发状态。后区（AP）是 NMOSD 最常见的受累部位之一，在后区可能在 NMOSD 的发病机制和临床异质性方面具有特殊意义。我们的研究旨在探讨 AP 起始 NMOSD 患者的临床和复发特征。
对根据 2015 年 IPND 标准确定的 166 例 AQP4-IgG 阳性 NMOSD 患者进行回顾性研究。根据首发症状部位，将患者分为 AP 起始（APO-NMOSD）组和非 AP 起始（NAPO-NMOSD）组。比较两组的临床特征和复发差异。
APO-NMOSD 组和 NAPO-NMOSD 组的人群比例为 24:142。APO-NMOSD 患者年龄较小（34.6y 与 42.3y，P = 0.013），首发时的 EDSS 较低（0.7 与 4.2，p = 0.028）和末次随访时的 EDSS 较低（1.9 与 3.3，p = 0.001），首发时更有可能出现多灶性病变（33.3%与 9.2%，P = 0.001）。此外，他们的年复发率更高（0.4 ± 0.28 与 0.19 ± 0.25，P = 0.012）。在未经免疫治疗的自然病程 NMOSD 患者中，APO-NMSOD 的首次复发时间更短（P < 0.001），年复发率更高（0.31 ± 0.22 与 0.16 ± 0.26，P = 0.038）。与视神经炎起始-NMOSD（HR 2.641，95%CI 1.427-4.887，p = 0.002）和脊髓炎起始-NMOSD 组（HR 3.593，95%CI 1.736-7.438，p = 0.001）相比，APO-NMOSD 患者首次复发时发生复发的风险更高。与 NAPO-NMOSD 相比，APO-NMOSD 在首次复发时更有可能出现脑干复发（28.6%与 4.7%，p<0.001），而 NAPO-NMOSD 更易发生视神经受累（10.7%与 41.1%，p = 0.01）。
AQP4-IgG 阳性 NMOSD 患者伴有 AP 起始表现为更年轻和更高的复发风险。临床医生应注意 NMOSD 中的 AP 损伤，因为这可能表明存在复发的潜在风险。
回顾性注册。
