title: Validation of Clinicoradiological Criteria for the Diagnosis of Cerebral Amyloid Angiopathy-Related Inflammation.
abstract: OBJECTIVE
  Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an important diagnosis to reach in clinical practice because many patients with the disease respond to immunosuppressive therapy. Reliable noninvasive diagnostic criteria for CAA-ri would allow some patients to avoid the risk of brain biopsy.
  
  
  OBJECTIVE
  To test the sensitivity and specificity of clinical and neuroimaging-based criteria for CAA-ri.
  
  
  METHODS
  We modified the previously proposed clinicoradiological criteria and retrospectively analyzed clinical medical records and magnetic resonance imaging fluid-attenuated inversion recovery and gradient-echo scans obtained from individuals with CAA-ri and noninflammatory CAA. At 2 referral centers between October 1, 1995, and May 31, 2013, and between January 1, 2009, and December 31, 2011, participants included 17 individuals with pathologically confirmed CAA-ri and 37 control group members with pathologically confirmed noninflammatory CAA. The control group was further divided into those with past lobar intracerebral hemorrhage (ICH) (n = 21) and those with cerebral microbleeds only and no history of ICH (n = 16). The dates of our analysis were September 1, 2012, to August 31, 2015.
  
  
  METHODS
  The sensitivity and specificity of prespecified criteria for probable CAA-ri (requiring asymmetric white matter hyperintensities extending to the subcortical white matter) and possible CAA-ri (not requiring the white matter hyperintensities to be asymmetric).
  
  
  RESULTS
  The 17 patients in the CAA-ri group were a mean (SD) of 68 (8) years and 8 (47%) were women. In the CAA-ri group, 14 of 17 (82%) met the criteria for both probable and possible CAA-ri. In the control group having noninflammatory CAA with lobar ICH, 1 of 21 (5%) met the criteria for possible CAA-ri, and none met the criteria for probable CAA-ri. In the control group having noninflammatory CAA with no ICH, 11 of 16 (69%) met the criteria for possible CAA-ri, and 1 of 16 (6%) met the criteria for probable CAA-ri. These findings yielded a sensitivity and specificity of 82% and 97%, respectively, for the probable criteria and a sensitivity and specificity of 82% and 68%, respectively, for the possible criteria.
  
  
  CONCLUSIONS
  Our data suggest that a reliable diagnosis of CAA-ri can be reached from basic clinical and magnetic resonance imaging information alone, with good sensitivity and excellent specificity.
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston2The Inflammatory Cerebral Amyloid Angiopathy and Alzheimer's Disease ßiomarkers International Network, University of M.
authors.forename: Eitan
authors.initials: E
authors.lastname: Auriel
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Andreas
authors.initials: A
authors.lastname: Charidimou
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: M Edip
authors.initials: ME
authors.lastname: Gurol
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Jun
authors.initials: J
authors.lastname: Ni
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Ellis S
authors.initials: ES
authors.lastname: Van Etten
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Sergi
authors.initials: S
authors.lastname: Martinez-Ramirez
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Gregoire
authors.initials: G
authors.lastname: Boulouis
authors.affiliation: The Inflammatory Cerebral Amyloid Angiopathy and Alzheimer's Disease ßiomarkers International Network, University of Milano-Bicocca, Monza, Italy3Milan Center for Neuroscience, School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Piazza
authors.affiliation: The Inflammatory Cerebral Amyloid Angiopathy and Alzheimer's Disease ßiomarkers International Network, University of Milano-Bicocca, Monza, Italy3Milan Center for Neuroscience, School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.
authors.forename: Jacopo C
authors.initials: JC
authors.lastname: DiFrancesco
authors.affiliation: Neuropathology Service, C. S. Kubik Laboratory for Neuropathology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Matthew P
authors.initials: MP
authors.lastname: Frosch
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Oct Vio M
authors.initials: OV
authors.lastname: Pontes-Neto
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston5Population Health Research Institute, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Ashkan
authors.initials: A
authors.lastname: Shoamanesh
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Yael
authors.initials: Y
authors.lastname: Reijmer
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Anastasia
authors.initials: A
authors.lastname: Vashkevich
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Alison M
authors.initials: AM
authors.lastname: Ayres
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Kristin M
authors.initials: KM
authors.lastname: Schwab
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston.
authors.forename: Anand
authors.initials: A
authors.lastname: Viswanathan
authors.affiliation: The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston2The Inflammatory Cerebral Amyloid Angiopathy and Alzheimer's Disease ßiomarkers International Network, University of M.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Greenberg
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016657:Cerebral Amyloid Angiopathy / Q000000981:diagnostic imaging* / Q000473:pathology*; D002543:Cerebral Hemorrhage / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006801:Humans; D007249:Inflammation / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012189:Retrospective Studies