@article {Wintermark2172, author = {M. Wintermark and N.K. Hills and G.A. DeVeber and A.J. Barkovich and T.J. Bernard and N.R. Friedman and M.T. Mackay and A. Kirton and G. Zhu and C. Leiva-Salinas and Q. Hou and H.J. Fullerton and the VIPS Investigators}, editor = {, and Dowling, Michael M. and Benedict, Susan L. and Bernard, Timothy J. and Fox, Christine K. and DeVeber, Gabrielle A. and Friedman, Neil R. and Lo, Warren D. and Ichord, Rebecca N. and Tan, Marilyn A. and Mackay, Mark T. and Kirton, Adam and Hernandez-Chavez, Marta I. and Humphreys, Peter and Jordan, Lori C. and Sultan, Sally and Rivkin, Michael J. and Rafay, Mubeen F. and Titomanlio, Luigi and Kovacevic, Gordana S. and Yager, Jerome Y. and Amlie-Lefond, Catherine and Dlamini, Nomazulu and Condie, John and Yeh, Ann and Kneen, Rachel and Bjornson, Bruce and Pergami, Paola and Zou, Li Ping and Elbers, Jorina M. and Abdalla, Abdalla and Chan, Anthony K. and Farooq, Osman and Lim, Mingming J. and Carpenter, Jessica L. and Pavlakis, Steven and Wong, Virginia C. and Forsyth, Robert}, title = {Clinical and Imaging Characteristics of Arteriopathy Subtypes in Children with Arterial Ischemic Stroke: Results of the VIPS Study}, volume = {38}, number = {11}, pages = {2172--2179}, year = {2017}, doi = {10.3174/ajnr.A5376}, publisher = {American Journal of Neuroradiology}, abstract = {BACKGROUND AND PURPOSE: Childhood arteriopathies are rare but heterogenous, and difficult to diagnose and classify, especially by nonexperts. We quantified clinical and imaging characteristics associated with childhood arteriopathy subtypes to facilitate their diagnosis and classification in research and clinical settings.MATERIALS AND METHODS: The Vascular Effects of Infection in Pediatric Stroke (VIPS) study prospectively enrolled 355 children with arterial ischemic stroke (2010{\textendash}2014). A central team of experts reviewed all data to diagnose childhood arteriopathy and classify subtypes, including arterial dissection and focal cerebral arteriopathy{\textendash}inflammatory type, which includes transient cerebral arteriopathy, Moyamoya disease, and diffuse/multifocal vasculitis. Only children whose stroke etiology could be conclusively diagnosed were included in these analyses. We constructed logistic regression models to identify characteristics associated with each arteriopathy subtype.RESULTS: Among 127 children with definite arteriopathy, the arteriopathy subtype could not be classified in 18 (14\%). Moyamoya disease (n = 34) occurred mostly in children younger than 8 years of age; focal cerebral arteriopathy{\textendash}inflammatory type (n = 25), in children 8{\textendash}15 years of age; and dissection (n = 26), at all ages. Vertigo at stroke presentation was common in dissection. Dissection affected the cervical arteries, while Moyamoya disease involved the supraclinoid internal carotid arteries. A banded appearance of the M1 segment of the middle cerebral artery was pathognomonic of focal cerebral arteriopathy{\textendash}inflammatory type but was present in \<25\% of patients with focal cerebral arteriopathy{\textendash}inflammatory type; a small lenticulostriate distribution infarct was a more common predictor of focal cerebral arteriopathy{\textendash}inflammatory type, present in 76\%. It remained difficult to distinguish focal cerebral arteriopathy{\textendash}inflammatory type from intracranial dissection of the anterior circulation. We observed only secondary forms of diffuse/multifocal vasculitis, mostly due to meningitis.CONCLUSIONS: Childhood arteriopathy subtypes have some typical features that aid diagnosis. Better imaging methods, including vessel wall imaging, are needed for improved classification of focal cerebral arteriopathy of childhood.FCAfocal cerebral arteriopathy of childhoodFCA-dfocal cerebral arteriopathy{\textendash}dissection typeFCA-ifocal cerebral arteriopathy{\textendash}inflammatory typeTCAtransient cerebral arteriopathyVIPSVascular Effects of Infection in Pediatric Stroke}, issn = {0195-6108}, URL = {https://www.ajnr.org/content/38/11/2172}, eprint = {https://www.ajnr.org/content/38/11/2172.full.pdf}, journal = {American Journal of Neuroradiology} }