Objective

To describe the clinical characteristics of neuro-Behçet’s syndrome (NBS) and to define the factors associated with relapses and poor outcome.

Methods

Among 2118 patients with Behçet’s syndrome who fulfilled the international study group criteria, 208 (9.8%) patients had NBS. Retrospective data of 125 NBS patients (55.5% male; mean age 37.2 ± 11.8 years) were analysed. We divided patients into two subgroups, either parenchymal (p-NBS) or non-parenchymal (np-NBS), according to international consensus recommendations for NBS. We assessed the predictor factors associated with relapse and poor outcome—which was defined as a modified Rankin score (mRS) ≥ 3 at last follow-up and/or death—using Cox and logistic regression analyses, respectively.

Results

In total, 79 (63.2%) patients presented with p-NBS and 46 (36.8%) presented with np-NBS. Ocular involvement was more common in p-NBS than np-NBS (55.7% vs. 37.0%, p = 0.04), whereas vascular involvement excluding cerebral vein thrombosis was more frequent in patients with np-NBS (19.0% vs. 52.2%, p < 0.001). Forty-two patients (33.6%) experienced at least one relapse. Factors associated with relapse were BS diagnosis at a younger age and cranial nerve dysfunction (HR 0.96 95% CI 0.93–0.99 and 2.36 95% CI 1.23–4.52, respectively). After a median of 68 (Q1–Q3: 25–125) months, 23 patients (18.4%) had a poor outcome. Indicators of a poor outcome were higher initial mRS and the progressive p-NBS type (OR 8.28 95% CI 1.04–66.20 and 33.57 95% CI 5.99–188.21, respectively).

Conclusion

Our findings indicate that clinical characteristics and prognosis differ between NBS subgroups, of which patients with p-NBS have worse outcomes.

中文翻译：

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神经白塞综合征复发和预后不良的预后因素：来自单个中心的临床长期随访结果

客观的

描述神经白塞综合征 (NBS) 的临床特征并确定与复发和不良结果相关的因素。

方法

在符合国际研究组标准的 2118 名白塞综合征患者中， 208 名（9.8%）患者患有 NBS。分析了 125 名 NBS 患者（55.5% 男性；平均年龄 37.2 ± 11.8 岁）的回顾性数据。根据 NBS 的国际共识建议，我们将患者分为两个亚组，即实质 (p-NBS) 或非实质 (np-NBS)。我们分别使用 Cox 和逻辑回归分析评估了与复发和不良结果相关的预测因素——其定义为最后一次随访和/或死亡时的改良 Rankin 评分 (mRS) ≥ 3。

结果

总共有 79 名 (63.2%) 患者出现 p-NBS，46 名 (36.8%) 患者出现 np-NBS。p-NBS 患者的眼部受累比 np-NBS 更常见（55.7% 对 37.0%，p  = 0.04），而 np-NBS 患者中不包括脑静脉血栓形成的血管受累更常见（19.0% 对 52.2%，p  < 0.001)。42 名患者 (33.6%) 经历了至少一次复发。与复发相关的因素是年龄较小的 BS 诊断和颅神经功能障碍（分别为 HR 0.96 95% CI 0.93-0.99 和 2.36 95% CI 1.23-4.52）。在中位 68 个月（Q1-Q3：25-125）个月后，23 名患者（18.4%）的结果不佳。不良结果的指标是较高的初始 mRS 和进行性 p-NBS 类型（OR 8.28 95% CI 1.04-66.20 和 33.57 95% CI 5.99-188.21，分别）。

结论

我们的研究结果表明，NBS 亚组之间的临床特征和预后不同，其中 p-NBS 患者的预后较差。