Chamoun Nadia, Elbejjani Martine, El Ayoubi Nabil K, Hatab Taha, Hazimeh Dana, Ibrahim Michael, Merashli Mira
Department of Internal Medicine, American University of Beirut Medical Center, Beirut 1107 2020, Lebanon.
Clinical Research Institute, Faculty of Medicine, American University of Beirut Medical Center, Beirut 1107 2020, Lebanon.
J Clin Med. 2025 Apr 8;14(8):2543. doi: 10.3390/jcm14082543.
: Behçet's Disease (BD) is a complex vasculitis affecting multiple organ systems, with Neuro-Behçet's Disease (NBD) representing a rare yet severe manifestation. Data on NBD are limited, particularly in Middle Eastern populations. : This retrospective observational study, spanning from 2000 to 2021, involved 262 BD patients at a tertiary medical center in Lebanon. NBD was diagnosed based on International Consensus Recommendation diagnostic criteria. Clinical data, including demographics, manifestations, inflammatory blood markers, genetics, and treatments, were collected. The modified Rankin Scale (mRS) was used to assess disease severity. : Among the cohort, 27 (10.3%) had NBD, with headaches, weakness, and dizziness as the most common presenting symptoms. The prevalence of NBD was similar across genders, which differs from some regional studies. HLA-B51 positivity was found in 50 out of 60 (83.3%) tested BD patients. Parenchymal NBD cases exhibited greater disease severity than non-parenchymal cases, with female patients experiencing a more severe course compared to males. Elevated inflammatory markers (CRP and ESR) were more common in patients with severe NBD. Corticosteroids and colchicine were the most commonly used therapies overall, while patients with better disease severity were more frequently prescribed methotrexate, mycophenolate, cyclophosphamide, adalimumab, and rituximab. An analysis of disease progression showed that at presentation, 57.1% ( = 12) of NBD patients had mild to moderate disability, which increased to 76.2% ( = 16) at the last follow-up, including 10 patients who showed an improvement in their mRS score. : This study provides valuable insights into the prevalence and clinical characteristics of NBD in a Middle Eastern population. These findings enhance our understanding of NBD in the Middle East, highlighting the need for further research to improve diagnosis and management.
白塞病（BD）是一种影响多个器官系统的复杂血管炎，神经白塞病（NBD）是一种罕见但严重的表现形式。关于NBD的数据有限，尤其是在中东人群中。：这项回顾性观察研究涵盖了2000年至2021年期间黎巴嫩一家三级医疗中心的262例BD患者。NBD根据国际共识推荐诊断标准进行诊断。收集了包括人口统计学、临床表现、炎症血液标志物、遗传学和治疗方法等临床数据。采用改良Rankin量表（mRS）评估疾病严重程度。：在该队列中，27例（10.3%）患有NBD，头痛、乏力和头晕是最常见的首发症状。NBD的患病率在男女之间相似，这与一些地区研究不同。在60例接受检测的BD患者中，有50例（83.3%）HLA - B51呈阳性。实质性NBD病例的疾病严重程度高于非实质性病例，女性患者的病程比男性更严重。炎症标志物（CRP和ESR）升高在重度NBD患者中更为常见。皮质类固醇和秋水仙碱是总体上最常用的治疗方法，而疾病严重程度较好的患者更常使用甲氨蝶呤、霉酚酸酯、环磷酰胺、阿达木单抗和利妥昔单抗。疾病进展分析显示，就诊时，57.1%（n = 12）的NBD患者有轻度至中度残疾，在最后一次随访时增加到76.2%（n = 16），其中10例患者的mRS评分有所改善。：这项研究为中东人群中NBD的患病率和临床特征提供了有价值的见解。这些发现增进了我们对中东地区NBD的理解，强调了进一步研究以改善诊断和管理的必要性。
