PT  - JOURNAL ARTICLE
AU  - Suthiphosuwan, S.
AU  - Bharatha, A.
AU  - Hsu, C.C.-T.
AU  - Lin, A.W.
AU  - Maloney, J.A.
AU  - Munoz, D.G.
AU  - Palmer, C.A.
AU  - Osborn, A.G.
TI  - Tumefactive Primary Central Nervous System Vasculitis: Imaging Findings of a Rare and Underrecognized Neuroinflammatory Disease
AID  - 10.3174/ajnr.A6736
DP  - 2020 Nov 01
TA  - American Journal of Neuroradiology
PG  - 2075--2081
VI  - 41
IP  - 11
4099  - http://www.ajnr.org/content/41/11/2075.short
4100  - http://www.ajnr.org/content/41/11/2075.full
SO  - Am. J. Neuroradiol.2020 Nov 01; 41
AB  - 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.PCNSVprimary central nervous system vasculitist-PCNSVtumefactive PCNSVABRAamyloidβ–associated angiitisCAA-RIcerebral amyloid angiopathy–related inflammationESRerythrocyte sedimentation rateMRPMR perfusionCRPC-reactive proteinPCRpolymerase chain reactionVWIvessel wall imagingPCNSLprimary CNS lymphoma