Background/Aim: Neurological symptoms (neuroBehcet's disease; NBD) occur in a fraction of Behcet's disease (BD) patients and often present with parenchymal brain lesions and clinical exacerbations. Our aim was to identify genes associated with attack and remission periods of NBD. Materials and Methods: Microarray analysis was performed using peripheral blood mononuclear cell (PBMC) samples obtained during attack and remission periods of five NBD patients. Expression levels of the most significantly upregulated genes were measured with real-time PCR using PBMC samples of 15 NBD patients and 20 healthy controls. Results: During NBD attacks, the most remarkably upregulated genes were defensin alpha 1B (DEFA1B) and NLR family, pyrin domain containing 3 (NLRP3). Real time PCR studies showed significantly increased DEFA1B and NLRP3 expression levels during attacks. Conclusion: Immunological factors showing the most significant increase in expression during NBD attacks were primarily associated with innate immunity functions. DEFA1B and NLRP3 can be used as biomarkers for estimation of disease activity in NBD.