Primary Central Nervous System Vasculitis as an Unusual Cause of Intracerebral Hemorrhage: A Case Report

A 64-year-old male with a history of transverse myelitis presented to the hospital with a decreased level of consciousness of one day's duration. CT of the head revealed intracranial hemorrhage measuring 2 x 1.2 cm in the right temporal lobe and multiple small hemorrhages in the left hemisphere, suggestive of vasculitis. Initial vasculitis workup was negative for antinuclear antibody (ANA), complement component 3 (C3), and antineutrophil cytoplasmic antibodies: P-ANCA, C-ANCA. Syphilis, hepatitis B and C, West Nile virus antibody [immunoglobulin G (IgG) and immunoglobulin M (IgM)], herpes simplex virus (HSV) polymerase chain reaction (PCR), and HIV 1 and 2 were also negative. In view of the CT scan findings suggestive of vasculitis and the vague presentation of primary central nervous system vasculitis (PCNSV), a brain biopsy was performed. It revealed angiocentric granulomatous inflammation with focal vessel disruption and associated parenchymal hemorrhage, consistent with a diagnosis of granulomatous vasculitis. The patient received levetiracetam, multiple high doses of steroids, and six cycles of cyclophosphamide for a six-month duration. After induction, he has remained in remission without any maintenance therapy until now (eight years post-presentation).