Neurological involvement of Behçet’s syndrome (BS), termed NBS, primarily presents as parenchymal NBS (p-NBS) or vascular NBS, mainly cerebral venous sinus thrombosis (CVST). Defining the clinical spectrum and prognostic determinants is essential for optimized management and outcome prediction.

Medical records of 2,640 patients followed at the Neuro-Behçet Outpatient Clinic, Istanbul University–Cerrahpasa, between 1983 and 2024 were reviewed. Among them, 507 patients who fulfilled BS criteria and were definitively diagnosed with NBS were included. Clinical, radiological, and laboratory data were analyzed to identify prognostic factors and treatment-related outcomes.

The study included 507 patients (365 males, 142 females; M/F 2.57; median age 33.2 ± 24.8 years). At presentation, 155 patients had isolated neurological symptoms, while 352 reported multiple complaints. Motor weakness and dysarthria at onset were associated with poorer outcomes, whereas isolated headache correlated with lower disability at last follow-up (last EDSS). CVST was associated with a more favorable course, while myelitis predicted higher disability and increased relapse risk. Patients with lower disability at last follow-up more often had CVST and fewer relapses. Infliximab use was associated with fewer relapses and lower last EDSS at follow-up. 

In this large single-center cohort, initial disability, relapse pattern, and infliximab therapy emerged as key predictors of long-term outcomes. These results support the importance of early recognition and aggressive treatment in high-risk subgroups to prevent irreversible neurological disability.