Primary angiitis of the central nervous system: report of eight cases from a single Italian center

Abstract

The primary angiitis of the central nervous system (PACNS) is a rare and potentially fatal form of vasculitis with unknown etiology. Headache and encephalopathy are the most frequent symptoms. Neuroimaging plays an important role in the diagnosis, but the pattern of abnormal findings is not specific. In some cases brain biopsy is mandatory. PACNS is often described as a diagnostic and therapeutic challenge for clinicians considering the lack of univocal diagnostic criteria and paucity of studies evaluating the long-term outcome. This brief work reports how we managed eight patients with PACNS from diagnosis to long-term follow up treatment. Headache and focal acute neurological deficits were the most common symptoms. Magnetic resonance imaging (MRI) was abnormal in all patients with different patterns of infarctions or intraparenchymal/subarachnoid hemorrhages or their combination. Cerebral angiography demonstrated pathological findings compatible with vasculitis in all cases. Other causes of encephalopathy were ruled out. Patients with severe clinical and neuroradiological findings were treated with steroids and immunosuppressive therapy using cyclophosphamide/methotrexate. Patients with less aggressive PACNS, were treated with steroids only. In one case with multiple relapses infliximab was used after first-line immunosuppressive therapy failure. PACNS suspicion is based on the combination of demographic/clinical and MRI findings and the exclusion of other causes of multifocal encephalopathy. A positive angiography has a diagnostic value in an adequate clinical field. A strict collaboration of neurologists, neuroradiologists, and immunoreumatologists is essential in the management of PACNS both in the diagnostic and therapeutic phases.