RT Journal Article
SR Electronic
T1 Tumefactive Primary Central Nervous System Vasculitis: Imaging Findings of a Rare and Underrecognized Neuroinflammatory Disease
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 2075
OP 2081
DO 10.3174/ajnr.A6736
VO 41
IS 11
A1 Suthiphosuwan, S.
A1 Bharatha, A.
A1 Hsu, C.C.-T.
A1 Lin, A.W.
A1 Maloney, J.A.
A1 Munoz, D.G.
A1 Palmer, C.A.
A1 Osborn, A.G.
YR 2020
UL http://www.ajnr.org/content/41/11/2075.abstract
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