The Role of Catheter Angiography in the Diagnosis of Central Nervous System Vasculitis

Abstract

Background: Central nervous system vasculitis (CNSV) is a rare disorder, the pathophysiology of which is not fully understood. It involves a combination of inflammation and thrombosis. CNSV is most commonly associated with headache, gradual changes in mental status, and focal neurological symptoms. Diagnosis requires the effective use of history, laboratory testing, imaging, and biopsy. Catheter angiography can be a powerful tool in the diagnosis when common and low-frequency angiographic manifestations of CNSV are considered. We review these manifestations and their place in the diagnostic algorithm of CNSV.

Summary: We reviewed the PubMed database for case series of CNSV that included 5 or more patients. Demographic and angiographic findings were collected. Angiographic findings were dichotomized between common and low-frequency findings. A system for incorporating these findings into clinical decision-making is proposed.

Key message: CNSV is a diagnostic challenge due to the absence of a true gold standard test. In the absence of such a test, catheter angiography remains a central piece of the diagnostic puzzle when appropriately employed and interpreted.

Keywords: Angiitis; Angiography; Primary central nervous system vasculitis; Vasculitis.

Fig. 1

Illustration of autopsy findings of inflammatory vascular changes included in the early angiographic paper by Hinck et al. [12].

Fig. 2

Frequency of use of angiographic descriptors among the 25 articles identified as meeting the search criteria. * Beading, alternating stenosis and dilation, alternating stenosis and ectasia, and a ‘sausage’ pattern were considered synonyms of segmental stenosis. ** Attenuation of distal arterial branches. SC ICA = Supraclinoid internal carotid artery.

Fig. 3

A flow diagram illustrating a systematic approach to the workup of possible CNSV.