Neuro-Behçet’s disease (NBD) is a rare but serious manifestation of Behçet’s disease, affecting 5% of patients. It involves multifocal central nervous system inflammation and venous thrombosis. Diagnosis is often delayed due to its rarity, unfamiliar neuroimaging patterns, and absence of definitive biomarkers. This case highlights classic imaging features and the importance of early immunosuppressive therapy.

A 21-year-old African American man presented with a five-year history of intermittent headaches, blurry vision in the right eye, recurrent cerebral venous sinus thrombosis (CVST), and controlled seizures. MRI of the brain 6 months prior to presentation revealed T2-FLAIR hyperintensity in the right pons, cerebral peduncle, gangliocapsular region, insula, mesial temporal lobe, and optic pathways. MRV showed extensive sinus thrombosis with collateral venous formation. Neurological exam revealed mild left hemiparesis and hyperreflexia. Ophthalmology evaluation was negative for uveitis or papillitis. Three months later, repeat MRI showed progression with new enhancement in the right thalamus, midbrain, and pons. The patient also had a history of recurrent oral ulcers, right ventricular cardiac thrombosis, and extensive pulmonary embolism; features supportive of Behçet’s disease. CSF analysis showed lymphocytic pleocytosis and elevated protein. Hypercoagulable and rheumatologic serologic evaluation was negative, including absent HLA-B51. Before rheumatology consultation, he developed refractory status epilepticus and repeat imaging showed worsening bilateral T2 FLAIR hyperintensities. Treatment with IV steroids and high-dose prednisone was ineffective. Based on clinical history, imaging and the 2014 International Criteria for Behçet’s disease, severe NBD was diagnosed. Treatment with infliximab and azathioprine led to marked clinical recovery and near-complete resolution of MRI abnormalities within four months.