Clinical, radiologic and prognostic features in neurologic involvement of Behçet's disease

Introduction and Objective: The neurologic involvement of Behçet's syndrome (BS) is called Neuro-Behçet's syndrome (NBS). Direct neurologic involvement of BS can be divided into two groups: 1) parenchymal (p-NBS) involvement; 2) vascular involvement, cerebral venous sinus thrombosis (CVST). In our study, we aimed (1) to evaluate the effect of different clinical and laboratory features and localization of structural lesions on the prognosis; (2) to determine the role of imaging findings in diagnosing the disease; and (3) to investigate the relationship between clinical features and disease progression. Methods: We retrospectively reviewed the medical records of 2640 patients admitted to the Neuro-Behçet's outpatient clinic of the Department of Neurology, Istanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Neurology (1983-2024) and analysed the data of 507 patients who fulfilled the criteria for Behçet's Disease and were definitively diagnosed with NBS. In this observational study, the medical records were retrospectively analysed, and no intervention was performed during the diagnosis and follow-up period, as well as in treatment choices. Results: 507 patients (M:365 F:142, M/F ratio: 2.57, median age 33.2 ± 24.8 years) were included in the study. 154 patients presented to the neurology clinic with an isolated neurologic complaint, while 351 patients had two or more complaints. During follow-up, initial and final EDSS scores were lower in patients presenting with headache and higher in the group with multiple attacks. Patients presenting with motor weakness and dysarthria had a worse clinical course, while patients presenting with isolated headache had lower disability scores. Patients with CVST showed a better clinical course compared to the other groups, whereas myelitis was associated with a poorer outcome. Patients with low EDSS scores at the last follow-up had a higher incidence of CVST and less attack recurrence. The risk of recurrent attacks was lower in the group receiving infliximab and this group had lower disability scores at the last follow-up. Conclusion: In our study, all known clinical and radiological involvement features of NBS were sub-grouped and in addition to this, the effect of Infliximab treatment, which is a more commonly used agent in recent years, on the development of clinical disability in these groups was evaluated. Among the many parameters compared with each other in our study, the model created with the parameters of initial EDSS score, presence of recurrent attacks, use of infliximab after NBS and simultaneous diagnosis of BD-NBS predicted the final EDSS score with a performance of 80.3%. Key words: Neuro-Behçet's Syndrome, Behçet's Disease, multiple attacks, Infliximab