Journal of Vasculitis

When No More Investigations are Left: A Probable Case of Primary Angiitis of the Central Nervous System

Abstract

Minesh Patel, Mohamed Rashad Ramali*, Saad Ahmed, Edward Needham, Imtiaz Ahmed and Alasdair Coles

Primary angiitis of the CNS (PACNS) is a distinct form of cerebral vasculitis, the former being confined to the central nervous system (CNS) with no systemic involvement. Diagnosing PACNS is challenging due to its varied presentation and rarity. We describe a case highly suggestive of PACNS which despite extensive investigation could not be confirmed even on histology but responded to immunosuppression. We present a 69-year old man who presented to the emergency department (ED) with acute confusion. Initial magnetic resonance (MR) imaging of the head had shown ischaemic infarcts and haemorrhages. Cerebrospinal fluid (CSF) analysis and serological work-up had ruled out a systemic vasculitis and an infectious disease to account for the presentation. Computerised tomography (CT) angiography nor digital subtraction angiography had reported any evidence of vasculopathy. The brain biopsy had failed to show features of vasculitis. Although the diagnosis of PACNS was not definitive, immunotherapy was commenced. A significant cognitive improvement was noted and correlated by resolution of the initial imaging changes.