AD inflammation

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

Eitan Auriel, MD, MSc1,2; Andreas Charidimou, MD, PhD1; M. Edip Gurol, MD, MSc1; Jun Ni, MD1; Ellis S. Van Etten, MD1; Sergi Martinez-Ramirez, MD1; Gregoire Boulouis, MD1; Fabrizio Piazza, PhD2,3; Jacopo C. DiFrancesco, MD, PhD2,3; Matthew P. Frosch, MD, PhD4; Octάvio M. Pontes-Neto, MD, PhD1; Ashkan Shoamanesh, MD1,5; Yael Reijmer, PhD1; Anastasia Vashkevich, BA1; Alison M. Ayres, BA1; Kristin M. Schwab, BA1; Anand Viswanathan, MD, PhD1; Steven M. Greenberg, MD, PhD1,2

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JAMA Neurol. Published online December 28, 2015. doi:10.1001/jamaneurol.2015.4078

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ABSTRACT

ABSTRACT | INTRODUCTION | METHODS | RESULTS | DISCUSSION | CONCLUSIONS |ARTICLE INFORMATION | REFERENCES

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.