Multiple cerebral infarctions from nonbacterial thrombotic endocarditis mimicking cerebral vasculitis

Primary vasculitis of the central nervous system (PVCNS) is an uncommon dis order that can present with a variety of symptoms, making diagnosis and man agement difficult. We describe a case of cerebral infarction that occurred from nonbacterial thrombotic endocarditis (NBTE) and presented with clinica l and radiologic imaging features that suggested PVCNS, The patient was a 5 8-year-old woman with left hemiparesis, aphasia, and episodic confusion. Ma gnetic resonance imaging of the brain demonstrated multifocal lesions consi stent with infarction involving both cerebral hemispheres, and cerebral ang iography showed changes consistent with vasculitis, Although brain biopsy f indings were normal, the patient was treated for presumed vasculitis with c yclophosphamide and prednisone, Four months later respiratory Failure secon dary to polymicrobial pneumonia and adult respiratory distress syndrome dev eloped, and she died. Autopsy revealed multiple infarcts in the heart, lung s, right kidney, spleen, and brain. Multiple thrombotic platelet-fibrin veg etations consistent with NBTE were found on all cardiac valves. Examination of the brain revealed no evidence of active or healed vasculitis. Cerebral angiography may show findings that suggest vasculitis, but it is not diagn ostic, as several other conditions may cause similar changes. Nonbacterial thrombotic endocarditis may cause multiple cerebral infarctions and can be difficult to distinguish from vasculitis, as specific diagnostic tests for PVCNS are lacking.