Nadia Chamoun

Background: 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. Methods: 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. Results: 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% (n = 12) of NBD patients had mild to moderate disability, which increased to 76.2% (n = 16) at the last follow-up, including 10 patients who showed an improvement in their mRS score. Conclusions: 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.

Background Behçet Disease (BD) is a complex, multi-systemic vasculitis characterized by inflammatory processes in blood vessels. Neuro-Behçet's Disease (NBD) represents a rare yet debilitating manifestation of BD, demanding a comprehensive understanding of its clinical features, prevalence, and distinction between parenchymal and non-parenchymal involvement. Methods and Results A retrospective observational study was conducted, spanning the years 2000 to 2022, involving 262 patients diagnosed with BD at a tertiary medical center in Lebanon. Neurological involvement was diagnosed based on International Consensus Recommendation criteria. Patients were stratified into groups based on NBD severity and subtypes, and clinical data, including demographics, manifestations, and genetic factors collected. The modified Rankin Scale (mRS) was used to assess NBD severity. Among the BD cohort, 10.3% were diagnosed with NBD. Clinical manifestations in NBD included prevalent neurological symptoms, with headaches, weakness, and dizziness being most frequent. The study found a similar prevalence of NBD between genders, contrary to some regional studies. Interestingly, HLA B51 positivity was observed in 83.3% of patients tested which were only sixty. Further stratification revealed a higher proportion of severe disease in parenchymal NBD compared to non-parenchymal cases. Conclusion This study provides valuable insights into the prevalence and clinical characteristics of NBD in a Middle Eastern population. Despite challenges in diagnosis and limited prevalence, the findings underscore the need for ongoing research to enhance the understanding, diagnosis, and management of NBD. These insights contribute to the broader comprehension of BD, particularly in the context of the Middle East, emphasizing the significance of tailored approaches for effective patient care.