Neuro-behcet’s syndrome: epidemiology, mechanisms, and emerging strategies in diagnosis and treatment

Neuro-Behçet’s Syndrome (NBS) is a severe and heterogeneous neurological manifestation of Behcet’s Syndrome (BS), a systemic vasculitis with a distinct prevalence along the historic Silk Road. This review synthesizes current knowledge on epidemiology, pathophysiology, clinical spectrum, and emerging treatment strategies for NBS. Genetic predisposition, particularly the HLA B51 allele, coupled with environmental triggers contributes to an aberrant immune response characterized by immune mediated vasculitis, blood brain barrier disruption, and the release of pro inflammatory cytokines such as TNF-α, IL-6, and IL-17. Two primary subtypes of NBS are recognized: parenchymal NBS, manifesting as meningoencephalitis with brain-stem, basal ganglia, or white matter lesions; and non-parenchymal NBS, which predominantly involves vascular complications such as cerebral venous sinus thrombosis. Clinical presentations range from cranial neuropathies and pyramidal signs to cognitive decline and psychiatric disturbances, with diagnosis relying on a combination of clinical findings, cerebrospinal fluid analysis, and advanced neuroimaging techniques. Magnetic Resonance Imaging (MRI) remains the diagnostic modality of choice, revealing T2 weighted hyperintensities and diffusion abnormalities, while emerging techniques such as Diffusion Tensor Imaging (DTI) further elucidate white matter microstructural changes. Current therapeutic approaches emphasize early and aggressive immunosuppression using high dose corticosteroids, conventional immunosuppressants, and biologic agents such as TNF-α inhibitors and IL-6 receptor antagonists. Despite these advances, long term outcomes remain suboptimal, prompting ongoing research into novel biomarkers and targeted therapies. The evidence presented underscores the urgent need for a multidisciplinary approach to optimize diagnosis, management, and patient outcomes in NBS.