Background

Behcet’s disease (BD) is a multisystem autoimmune relapsing vasculitis with an almost unknown etiology involving both large and small vessels. The neurological involvement called neuro-Behcet’s disease (NBD) is rare. NBD can be responsible for tumor-like masses mimicking low-grade gliomas in only a few cases.

Methods

We report here the main characteristics, treatment, and outcome of 43 patients (4 personal cases and 39 patients from the literature) with a pseudotumoral presentation of NBD (PT NBD). We compared our findings with those of the classical form of NBD.

Results

The median age was 35.86 (12–59 years) years, with a male predominance (67.4%). PT NBD was the inaugural of the disease in 51.2% of cases. The neurological manifestations included headache (n = 31), pyramidal syndrome (n = 28), cerebellar syndrome (n = 5), behavioral changes (n = 5), and pseudobulbar signs (n = 2). Ophthalmologic examination revealed papilledema in 3 cases. On cerebral imaging, the most affected regions of the brain were the capsulothalamic region (n = 15, 37.5%) and the brainstem (n = 14, 35). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoral or infectious lesions. Treatment consisted of corticosteroids (n = 40, 93%) and immunosuppressive agents (n = 28, 65.11%), leading to complete clinical and imaging remission in 41.5% of patients.

Conclusion

PT NBD is a rare but life-threatening condition.

中文翻译：

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假瘤性神经白塞氏病：病例系列和文献综述

背景

白塞氏病 (BD) 是一种多系统自身免疫性复发性血管炎，其病因几乎未知，涉及大血管和小血管。称为神经白塞氏病 (NBD) 的神经系统受累很少见。仅在少数情况下，NBD 会导致类似低级别神经胶质瘤的肿瘤样肿块。

方法

我们在此报告 43 例 NBD 假瘤 (PT NBD) 患者（4 例个人病例和 39 例文献中的患者）的主要特征、治疗和结果。我们将我们的发现与 NBD 的经典形式进行了比较。

结果

中位年龄为 35.86（12-59 岁），其中男性占多数（67.4%）。51.2% 的病例中，PT NBD 是该病的始发者。神经系统表现包括头痛（n  = 31）、锥体综合征（n  = 28）、小脑综合征（n  = 5）、行为改变（n  = 5）和假球征（n  = 2）。眼科检查发现视乳头水肿3例。在脑成像中，受影响最严重的大脑区域是囊丘脑区域 ( n  = 15, 37.5%) 和脑干 ( n  = 14, 35)。组织学分析显示坏死病灶伴有血管周围炎症浸润，没有肿瘤或感染性病灶的迹象。治疗包括皮质类固醇 ( n  = 40, 93%) 和免疫抑制剂 ( n  = 28, 65.11%)，导致 41.5% 的患者临床和影像学完全缓解。

结论

PT NBD 是一种罕见但危及生命的疾病。