Abstract: Objective To investigate the clinical features, imaging features, treatment and prognosis in patients with parenchymal neuro-Behçet syndrome (p-NBS). Methods This retrospective study analyzed the clinical data of p-NBS patients admitted to Xuanwu Hospital of Capital Medical University from January 2013 to September 2020. The p-NBS patients were selected as observation group, and the age- and gender-matched Behçet syndrome (BS) patients without neuro-Behçet syndrome (NBS) as control group. The clinical characteristics, laboratory tests [erythrocyte sedimentation rate (ESR), CRP, fibrinogen], Behçet’s disease current activity form 2006 (BDCAF2006) score between the two groups were compared. Results A total of 22 patients were included in each group. Compared to the control group, the observation group had higher incidence of ocular involvement (31.8% vs. 4.5%, P =0.046), and higher BDCAF2006 score (4.5±0.9 vs. 2.8±0.9, P <0.001). The pons was the most frequently involved site on MRI. In the observation group with 13 males (59.1%) and a mean age of 34.8±9.9 years, pyramidal tract signs (12/22, 54.5%), muscle weakness (11/22, 50.0%) and ataxia (10/22, 45.5%) were the most common neurological symptoms, white cells counts in cerebrospinal fluid (CSF) increased in 11 patients (50.0%) and CSF protein level increased in 5 patients (22.7%). All

p-NBS patients were treated with glucocorticoids and immunosuppressive agents, 2 patients (9.1%)

died during the median follow-up of 24 months (3-72 months). Conclusions p-NBS was more common in young men, with various clinical manifestations. The pons was the most frequently involved site. Glucocorticoids and immunosuppressive agents were the main treatment agents. p-NBS patients had more ocular involvement and higher disease activity, compared to the BS patients without NBS.

Key words: Beh?et syndrome; Parenchymal neuro-Beh?et syndrome; Clinical characteristics

摘要： 目的 分析实质性神经白塞综合征（parenchymal neuro-Behçet syndrome，p-NBS）的临床表现、影像 学特征、治疗及预后。 方法 回顾性分析2013年1月-2020年9月首都医科大学宣武医院收治的p-NBS患者的临床资料，选 择年龄和性别与p-NBS匹配的无神经白塞综合征（neuro-Behçet syndrome，NBS）的白塞综合征（Behçet syndrome，BS）患者为对照组，比较p-NBS组与对照组之间临床特征、实验室指标（红细胞沉降率、 CRP、纤维蛋白原）、2006版白塞综合征疾病活动度量表（Behçet’s disease current activity form 2006， BDCAF2006）评分的差异。 结果 p-NBS组和对照组各纳入22例患者。与对照组相比，p-NBS组患者眼部受累的比例（31.8% vs. 4.5%，P =0.046）更高，BDCAF2006评分（4.5±0.9分 vs. 2.8±0.9分，P <0.001）更高。头颅MRI 检查显 示p-NBS最常见的受累部位是脑桥（45.5%，10/22）。22例p-NBS患者中，男性13例（59.1%），平均年龄 34.8±9.9岁；锥体束征（54.5%，12/22）、肌无力（50.0%，11/22）和共济失调（45.5%，10/22）是最 常见的神经系统症状；11例（50.0%）患者脑脊液中细胞总数增加，5例（22.7%）蛋白含量增加。所有 p-NBS患者均予以糖皮质激素、免疫抑制剂治疗，在24（3～72）个月的中位随访期中，2例（9.1%）死亡。 结论 p-NBS好发于青年男性，临床表现多样，最常见的受累部位是脑桥。糖皮质激素和免疫抑制剂 是主要的治疗方法。与无NBS的BS患者相比，更易出现眼部受累，疾病活动性更高。

关键词: 白塞综合征; 实质性神经白塞综合征; 临床特征