A rare manifestation of systemic lupus erythematosus (SLE) is cerebral venous sinus thrombosis (CVST), in which early diagnosis and aggressive therapy are of prime importance for favorable outcome. The pathogenesis of CVST is largely unknown, but it is thought to be caused by cerebral vasculitis, antiphospholipid antibodies or other conditions associated with enhanced coagulability. We describe two cases of SLE with CVST which were not associated with antiphospholipid antibodies. Both cases were treated with immunosuppressants (intravenous methylprednisolone and cyclophosphamide pulse therapy) and anticoagulant drugs (heparin and subsequent maintenance therapy with warfarin). There was a marked improvement of neurologic symptoms with the disappearance of thrombus in a follow-up MRI. The possibility of CVST should be considered in any patients with SLE who show neuropsychiatric manifestations.
Adult ; Anti-Inflammatory Agents, Steroidal/therapeutic use ; Anticoagulants/therapeutic use ; Brain/radiography ; Cyclophosphamide/therapeutic use ; Female ; Glucocorticoids, Synthetic/therapeutic use ; Heparin/therapeutic use ; Human ; Immunosuppressive Agents/therapeutic use ; Lupus Erythematosus, Systemic/*complications ; Magnetic Resonance Imaging ; Methylprednisolone/therapeutic use ; Middle Age ; Sinus Thrombosis, Intracranial/*complications/drug therapy/physiopathology/radiography ; Treatment Outcome ; Warfarin/therapeutic use