Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy-related inflammation

Eitan Auriel, Andreas Charidimou, M. Edip Gurol, Jun Ni, Ellis S. Van Etten, Sergi Martinez-Ramirez, Gregoire Boulouis, Fabrizio Piazza, Jacopo C. Di Francesco, Matthew P. Frosch, Octuvio M. Pontes-Neto, Ashkan Shoamanesh, Yael Reijmer, Anastasia Vashkevich, Alison M. Ayres, Kristin M. Schwab, Anand Viswanathan, Steven M. Greenberg*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

201 Scopus citations

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.

Original languageEnglish
Pages (from-to)197-202
Number of pages6
JournalJAMA Neurology
Volume73
Issue number2
DOIs
StatePublished - Feb 2016
Externally publishedYes

Funding

FundersFunder number
National Institute on AgingR01AG026484

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