Neuroradiologic Characteristics of Primary Angiitis of the Central Nervous System According to the Affected Vessel Size

INTRODUCTION: Magnetic resonance imaging (MRI) has an important impact in diagnosing primary angiitis of the central nervous system (PACNS). However, neuroradiologic findings may vary immensely, making an easy and definite diagnosis challenging.

METHODS: In this retrospective, single center study, we analyzed neuroradiologic findings of patients with PACNS diagnosed at our hospital between 2009 and 2014. Furthermore, we classified patients according to the affected vessel size and compared imaging characteristics between the subgroups.

RESULTS: Thirty-three patients were included (mean age 43 [±15.3] years, 17 females) in this study. Patients with positive angiographic findings were classified as either medium or large vessel PACNS and presented more ischemic lesions (p < 0.001) and vessel wall enhancement (p = 0.017) compared to patients with small vessel PACNS. No significant differences were detected for the distribution of contrast-enhancing lesions (parenchymal or leptomeningeal), hemorrhages, or lesions with mass effect. Twenty-five patients underwent brain biopsy. Patients with medium or large vessel PACNS were less likely to have positive biopsy results.

DISCUSSION: It is essential to differentiate between small and medium/large vessel PACNS since results in MRI, digital subtraction angiography and brain biopsy may differ immensely. Since image quality of MR scanners improves gradually and brain biopsy may often be nonspecific or negative, our results emphasize the importance of MRI/MRA in the diagnosis process of PACNS.