Abstract
SUMMARY: Primary central nervous system vasculitis (PCNSV) is a poorly understood neuroinflammatory disease of the CNS affecting the intracranial vasculature. Although PCNSV classically manifests as a multifocal beaded narrowing of the intracranial vessels, some patients may not have angiographic abnormalities. A rare subset of patients with PCNSV present with masslike brain lesions mimicking a neoplasm. In this article, we retrospectively review 10 biopsy-confirmed cases of tumefactive PCNSV (t-PCNSV). All cases of t-PCNSV in our series that underwent CTA or MRA were found to have normal large and medium-sized vessels. T-PCNSV had a variable MR imaging appearance with most cases showing cortical/subcortical enhancing masslike lesion (70%), often with microhemorrhages (80%). Diffusion restriction was absent in all lesions. In summary, normal vascular imaging does not exclude the diagnosis of t-PCNSV. Advanced imaging techniques including MR perfusion and MR spectroscopy failed to demonstrate specific findings for t-PCNSV but assisted in excluding neoplasm in the differential diagnosis. Biopsy remains mandatory for definitive diagnosis.
ABBREVIATIONS:
- PCNSV
- primary central nervous system vasculitis
- t-PCNSV
- tumefactive PCNSV
- ABRA
- amyloidβ–associated angiitis
- CAA-RI
- cerebral amyloid angiopathy–related inflammation
- ESR
- erythrocyte sedimentation rate
- MRP
- MR perfusion
- CRP
- C-reactive protein
- PCR
- polymerase chain reaction
- VWI
- vessel wall imaging
- PCNSL
- primary CNS lymphoma
- © 2020 by American Journal of Neuroradiology