Neuro-Behçet's Disease

Abstract

Behçet’s disease (BD) is a chronic inflammatory disorder affecting multiple systems in the body. Neuro-Behçet’s disease (NBD) is the neurological manifestation of BD. It usually occurs late in the disease and tends to affect 10–30% of BD patients. The neurological symptoms may include cerebral thrombosis in the arterial or venous systems, meningoencephalitis, demyelination, memory loss, and behavioral changes. Although BD cases have been reported from all over the world, it is more common in the geographical regions on the historic Silk Route. The highest prevalence of the disease is in Turkey. It mostly affects individuals between 20 and 40years of age and affects men and women equally. BD etiology has not been well understood yet, but various genetic, environmental, and immunological factors are thought to play a role. Although the human leukocyte antigen (HLA)-B51 has long been suggested to be the dominant predisposing factor in BD, the non-major histocompatibility complex (MHC) class I genes have been associated with BD susceptibility as well. The genome-wide association studies (GWAS) reported various single-nucleotide polymorphism of certain genes in BD. Additionally, targeted next-generation sequencing methods have demonstrated non-synonymous variants (NSVs). The epigenetics (interspersed repetitive sequences, miRNAs) of BD and NBD have also been of interest to the researchers lately. Even though the studies primarily focus on BD rather than NBD, the data gathered for BD has been expected to be compatible with NBD etiopathogenesis. Further NBD-oriented epidemiologic, genetic, and epigenetic studies are needed to enlighten the disease etiology and to detect differential NBD biomarkers.