Primary angiitis of the central nervous system diagnosed post-mortem

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare vasculitis confined to the central nervous system (CNS) with no evidence of systemic inflammatory disease or infection. It usually occurs in patients in the 4th or 5th decades with a slight male predominance in adults. Patients often present with stroke, focal neurological defects, headache and cognitive impairment. The prognosis is variable, ranging from spontaneous regression to death. Diagnosis is based upon clinical, imaging and pathological diagnosis, in which histology is the gold standard. We present a case of a 59-year-old female, who despite extensive investigation, rapidly deteriorated with multi-territorial strokes in the course of a few months. At post-mortem, a range of acute and chronic changes consistent with PACNS were evident. In this case review, we will showcase the spectrum of changes seen in chronically untreated PACNS.