ANCA-associated vasculitis (AAV) causing bilateral cerebral infarction and subsequent intracerebral hemorrhage without renal and respiratory dysfunction.

A 56-year-old man complained of headache and the analgesia of the four extremities. Laboratory data presented signs of inflammation, but no other abnormality such as renal or respiratory dysfunction. Two months after the appearance of the primary symptoms, he developed cerebral infarction of the bilateral corona radiata. The patient did not have any of the risk factors for cerebrovascular disease. He was diagnosed with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) because the systemic examinations revealed only that he tested positive for anti-neutrophil cytoplasmic autoantibody (ANCA). Moreover, the biopsy of the sural nerve displayed microscopic polyangitis (MPA). Despite undergoing steroid pulse therapy, the patient died from the fatal cerebral hemorrhage. The clinical course of AAV is rapid progressive. In cases of atypical cerebrovascular disease, the possibility of ANCA should be investigated.