ABRA is a subtype of PCNSV (primary CNS vasculitis) with a beta pathology, found in about 18 % of biopsies showing vasculitis. PNSV has an incidence of about 2.4 per million. Presentation is cognitive impairment, +/- seizures, focal deficits, hallucinations, multifocal or confluent T2 white matter confluence and cortical microbleeds. Often CSF protein is elevated and there is a leukocytosis. This reported case had uveomeningeal findings clinically and on MRI.
Diagnosis by biopsy aND MAY BE AUTOIMMUNE AND RESPOND TO IMMUNOTHERAPY IN THIS CASE PREDNISONE 60/ DAY AND CELLCEPT 1500 BID RESULTED IN IMPROVEMENT
Neurology 2013; 81: 1596-1603
ABRA characteristics of patients: Authors from Mayo compared 78 patients, 40 with CAA (no inflammation) and 28 with granulomatous vasculitis (ABRA) , 10 with CAA related inflammation, and 118 matched patients with PCNSV with AB seen over 25 years.
ABRA patients were older, had more altered cognition, seizures and spells, gado positive leptomeninges, ICH, and higher CSF protein. Response to treatment was similar.