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. 2016 Feb;73(2):197-202.
doi: 10.1001/jamaneurol.2015.4078.

Validation of Clinicoradiological Criteria for the Diagnosis of Cerebral Amyloid Angiopathy-Related Inflammation

Eitan Auriel  1 , Andreas Charidimou  2 , M Edip Gurol  2 , Jun Ni  2 , Ellis S Van Etten  2 , Sergi Martinez-Ramirez  2 , Gregoire Boulouis  2 , Fabrizio Piazza  3 , Jacopo C DiFrancesco  3 , Matthew P Frosch  4 , Oct Vio M Pontes-Neto  2 , Ashkan Shoamanesh  5 , Yael Reijmer  2 , Anastasia Vashkevich  2 , Alison M Ayres  2 , Kristin M Schwab  2 , Anand Viswanathan  2 , Steven M Greenberg  1
Affiliations

Validation of Clinicoradiological Criteria for the Diagnosis of Cerebral Amyloid Angiopathy-Related Inflammation

Eitan Auriel et al. JAMA Neurol. 2016 Feb.

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.

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