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JAMA Neurol. 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.

Author information

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 ├čiomarkers 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 ├čiomarkers 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.

PMID:
26720093
DOI:
10.1001/jamaneurol.2015.4078
[Indexed for MEDLINE]

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