Due to our patient’s past history of sterile osteomyelitis, an autoinflammatory disorder, predisposing her to other autoinflammatory disorders [18], presenting clinical condition with SE and elevated inflammatory markers, including erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), vWF, and radiographic signs of vasculitis, while excluding other differential diagnoses, inflammatory brain diseases were investigated. The gene discussed is VWF; the disease is vasculitis.