Abstract：
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.
CONCLUSIONS: PT NBD is a rare but life-threatening condition.
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.
CONCLUSIONS: PT NBD is a rare but life-threatening condition.
摘要：
背景：Behcet病（BD）是一种多系统自身免疫性复发性血管炎，病因几乎未知，涉及大血管和小血管。神经参与称为神经Behcet病（NBD）是罕见的。仅在少数情况下，NBD可能是模仿低度神经胶质瘤的肿瘤样肿块的原因。
方法：我们在这里报告主要特征，治疗,以及43例患者（4例个人病例和39例来自文献）的NBD假瘤表现（PTNBD）的结果。我们将我们的发现与经典形式的NBD进行了比较。
结果：中位年龄为35.86（12-59岁），男性占主导地位（67.4%）。在51.2%的病例中，PTNBD是该病的首发。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），行为变化(n=5)，和假球征（n=2）。眼科检查发现3例乳头水肿。关于大脑成像，受影响最大的脑区是脑丘脑区（n=15，37.5％）和脑干（n=14，35）。组织学分析显示坏死病变伴血管周围炎症浸润，没有肿瘤或感染性病变的迹象。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%患者的临床和影像学完全缓解。
结论：PTNBD是一种罕见但危及生命的疾病。
方法：我们在这里报告主要特征，治疗,以及43例患者（4例个人病例和39例来自文献）的NBD假瘤表现（PTNBD）的结果。我们将我们的发现与经典形式的NBD进行了比较。
结果：中位年龄为35.86（12-59岁），男性占主导地位（67.4%）。在51.2%的病例中，PTNBD是该病的首发。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），行为变化(n=5)，和假球征（n=2）。眼科检查发现3例乳头水肿。关于大脑成像，受影响最大的脑区是脑丘脑区（n=15，37.5％）和脑干（n=14，35）。组织学分析显示坏死病变伴血管周围炎症浸润，没有肿瘤或感染性病变的迹象。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%患者的临床和影像学完全缓解。
结论：PTNBD是一种罕见但危及生命的疾病。
