Granulomatous angiitis of the central nervous system--case report

A 22-year-old male presented with granulomatous angiitis manifesting as headache. Computed tomography (CT) demonstrated a low-density area and ring-like enhancement in the right frontoparietal region. Steroid therapy caused rapid improvement in the signs and symptoms. Withdrawal of steroid was followed by recurrence of the headache and low-density area. The steroid treatment was resumed until he became asymptomatic 3 months after admission. He was followed up as an outpatient once every 2 weeks. One month after discharge, he complained of mild headache, and CT again demonstrated a mild increase in the low-density area. He was followed up continuously without steroid treatment. Two months after discharge, he suddenly lost consciousness, and CT demonstrated multiple intracerebral hematomas in the bilateral parietal regions. Removal of an intracerebral hematoma in the right parietal region and external decompression were carried out, but he died 3 days later. Histological examination of tissues from the right parietal cortex and leptomeninges demonstrated granulomatous inflammation with several giant cells in the vessels of the cerebral cortex and leptomeninges.