Eitan Auriel1, Andreas Charidimou2, M Edip Gurol2, Jun Ni2, Ellis S Van Etten2, Sergi Martinez-Ramirez2, Gregoire Boulouis2, Fabrizio Piazza3, Jacopo C DiFrancesco3, Matthew P Frosch4, Oct Vio M Pontes-Neto2, Ashkan Shoamanesh5, Yael Reijmer2, Anastasia Vashkevich2, Alison M Ayres2, Kristin M Schwab2, Anand Viswanathan2, Steven M Greenberg1. 1. 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. 2. The J. Philip Kistler Stroke Research Center, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston. 3. 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. 4. Neuropathology Service, C. S. Kubik Laboratory for Neuropathology, Massachusetts General Hospital and Harvard Medical School, Boston. 5. 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.
Abstract
IMPORTANCE: 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. DESIGN, SETTING, AND PARTICIPANTS: 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. MAIN OUTCOMES AND MEASURES: 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 AND RELEVANCE: 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.
IMPORTANCE: 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. DESIGN, SETTING, AND PARTICIPANTS: 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. MAIN OUTCOMES AND MEASURES: 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 AND RELEVANCE: 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: Andreas Charidimou; Gregoire Boulouis; M Edip Gurol; Cenk Ayata; Brian J Bacskai; Matthew P Frosch; Anand Viswanathan; Steven M Greenberg Journal: Brain Date: 2017-07-01 Impact factor: 13.501
Authors: Thanakit Pongpitakmetha; Panagiotis Fotiadis; Marco Pasi; Gregoire Boulouis; Li Xiong; Andrew D Warren; Kristin M Schwab; Jonathan Rosand; M Edip Gurol; Steven M Greenberg; Anand Viswanathan; Andreas Charidimou Journal: Neurology Date: 2020-04-13 Impact factor: 9.910
Authors: Oana M Dumitrascu; Erin M Okazaki; Steven H Cobb; Matthew A Zarka; Stephen A De Souza; Gyanendra Kumar; Cumara B O'Carroll Journal: Neuroophthalmology Date: 2017-09-19
Authors: Steven M Greenberg; Brian J Bacskai; Mar Hernandez-Guillamon; Jeremy Pruzin; Reisa Sperling; Susanne J van Veluw Journal: Nat Rev Neurol Date: 2019-12-11 Impact factor: 42.937