Abstract

Background: Behçet disease is an idiopathic chronic relapse multi-systemic vascular-inflammatory disease of unknown origin. Central nervous system involvement is considered one of the most severe manifestations of the disease, and it is categorized into primary or secondary. The primary neuro-Behçet disease can be sub-classified into parenchymal and non-parenchymal disease.

Objective: To identify neuro-Behçet disease clinical and radiological manifestations, and categorize the various presentations into characteristic patterns.

Patients and Methods: A case series study was conducted in Iraqi hospitals and outpatient clinics from March 2022 to December 2022. Demographic data of the patients and the duration of BD at the time of NBD diagnosis were recorded. The patients were clinically re-assessed for the fulfillment of Behçet disease diagnostic criteria. The enrolled patients were evaluated by neurologists in each province, for the manifestations of neuro-Behçet disease, whether current or previous presentation. Henceforth, a revision of patients' radiological reports was recorded.

Results: There were 80 patients in this study; the mean age of patients was 31.7 ± 9.1, with male predominance (62%). The mean duration of Behçet disease at neuro-Behçet diagnosis was 2.8 ± 2.4 years.

Regarding cumulative clinical manifestations, parenchymal symptoms were the most common, predominantly brainstem syndrome (67.5%). Non-parenchymal manifestations such as venous sinus thrombosis and pseudotumor cerebri presented in 46.3% of patients with Behçet disease. MRI findings reflected the clinical picture, with parenchymal involvement (55.1%) and brainstem hyperintensity being the most common (40%), followed by venous sinus thrombosis (43.8%).

Conclusion: Behçet disease neurological manifestations occurred mainly in men in their thirties to forties, and the majority of them presented within 2.8 years of Behçet disease onset. Brainstem involvement is the most common presentation. However, CVST pattern of the disease was prevalent in Iraq. Radiologically, brainstem hyperintensity is the most common finding in parenchymal NBD.