AbstractNeuro-Behçet’s disease (NBD) is subcategorized into parenchymal-NBD (P-NBD) and non-parenchymal-NBD types. Recently, P-NBD has been further subdivided into acute P-NBD (A-P-NBD) and chronic progressive P-NBD (CP-P-NBD). Although an increasing number of studies have reported the various clinical features of A-P-NBD and CP-P-NBD over the last two decades, there was a considerable inconsistency. Two investigators systematically searched four electrical databases to detect studies that provided sufficient data to assess the specific characteristics of A-P-NBD and CP-P-NBD. All meta-analysis was carried out by employing the random-model generic inverse variance method. We included 11 reports consisted of 184 A-P-NBD patients and 114 CP-P-NBD patients. While fever (42% for A-P-NBD, 5% for CP-P-NBD, p < 0.001, I² = 93%) was more frequently observed in A-P-NBD cases; sphincter disturbances (9%, 34%, P = 0.005, I² = 87%), ataxia (16%, 57%, P < 0.001, I² = 92%), dementia (7%, 61%, P < 0.001, I² = 97%), confusion (5%, 18%, P = 0.04, I² = 76%), brain stem atrophy on MRI (4%, 75%, P < 0.001, I² = 98%), and abnormal MRI findings in cerebellum (7%, 54%, P = 0.02, I² = 81%) were more common in CP-P-NBD. Cerebrospinal fluid cell count (94/mm³, 11/mm³, P = 0.009, I² = 85%) was higher in A-P-NBD cases. We demonstrated that A-P-NBD and CP-P-NBD had clearly different clinical features and believe that these data will help future studies investigating P-NBD.