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.
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。