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. 2024 May 28;102(10):e209386.
doi: 10.1212/WNL.0000000000209386. Epub 2024 May 6.

Boston Criteria v2.0 for Cerebral Amyloid Angiopathy Without Hemorrhage: An MRI-Neuropathologic Validation Study

Aaron R Switzer  1 , Andreas Charidimou  1 , Stuart McCarter  1 , Prashanthi Vemuri  1 , Aivi T Nguyen  1 , Scott A Przybelski  1 , Timothy G Lesnick  1 , Alejandro A Rabinstein  1 , Robert D Brown  1 , David S Knopman  1 , Ronald C Petersen  1 , Clifford R Jack Jr  1 , R Ross Reichard  1 , Jonathan Graff-Radford  1
Affiliations

Boston Criteria v2.0 for Cerebral Amyloid Angiopathy Without Hemorrhage: An MRI-Neuropathologic Validation Study

Aaron R Switzer et al. Neurology. .

Abstract

Background and objectives: Updated criteria for the clinical-MRI diagnosis of cerebral amyloid angiopathy (CAA) have recently been proposed. However, their performance in individuals without symptomatic intracerebral hemorrhage (ICH) presentations is less defined. We aimed to assess the diagnostic performance of the Boston criteria version 2.0 for CAA diagnosis in a cohort of individuals ranging from cognitively normal to dementia in the community and memory clinic settings.

Methods: Fifty-four participants from the Mayo Clinic Study of Aging or Alzheimer's Disease Research Center were included if they had an antemortem MRI with gradient-recall echo sequences and a brain autopsy with CAA evaluation. Performance of the Boston criteria v2.0 was compared with v1.5 using histopathologically verified CAA as the reference standard.

Results: The median age at MRI was 75 years (interquartile range 65-80) with 28/54 participants having histopathologically verified CAA (i.e., moderate-to-severe CAA in at least 1 lobar region). The sensitivity and specificity of the Boston criteria v2.0 were 28.6% (95% CI 13.2%-48.7%) and 65.3% (95% CI 44.3%-82.8%) for probable CAA diagnosis (area under the receiver operating characteristic curve [AUC] 0.47) and 75.0% (55.1-89.3) and 38.5% (20.2-59.4) for any CAA diagnosis (possible + probable; AUC 0.57), respectively. The v2.0 Boston criteria were not superior in performance compared with the prior v1.5 criteria for either CAA diagnostic category.

Discussion: The Boston criteria v2.0 have low accuracy in patients who are asymptomatic or only have cognitive symptoms. Additional biomarkers need to be explored to optimize CAA diagnosis in this population.

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Conflict of interest statement

A.R. Switzer, A. Charidimou, and S.J. McCarter report no disclosures relevant to the manuscript. P. Shemuri reports research support from the NIH. A. Nguyen, S.A. Przybelski, and T.G. Lesnick report no disclosures relevant to the manuscript. A.A. Rabinstein reports research support from the NIH. R.D. Brown reports no disclosures relevant to the manuscript. D.S. Knopman serves on a Data Safety Monitoring Board for the DIAN study; serves on a Data Safety Monitoring Board for a tau therapeutic for Biogen but receives no personal compensation; is a site investigator in the Biogen aducanumab trials; is an investigator in a clinical trial sponsored by Lilly Pharmaceuticals and the University of Southern California; and serves as a consultant for Samus Therapeutics, Roche, and Alzeca Biosciences but receives no personal compensation. RC Petersen serves as a consultant for Roche, Inc., Genentech Inc., Nestle, Inc., Eli Lilly and Co., and Eisai, Inc. and receives publishing royalties from Mild Cognitive Impairment (Oxford University Press, 2003) and UpToDate. C.R. Jack receives research support from the NIH. R.R. Reichard receives research support from the NIH. J. Graff-Radford serves on the DSMB for STROKENET and receives research support from the NIH. Go to Neurology.org/N for full disclosures.

Figures

Figure
Figure. Sensitivity Analysis Comparing the Number of PVSs Between 2 Different MR Sequences for Each Patient
PVSs were counted in the centrum semiovale on both T2-FSE (orange) and T1-weighted sequences (blue) for each patient. T1-weighted sequences frequently underreported the number of PVS compared with the amount counted on T2-FSE. FSE = fast spin echo; PVS = perivascular space.
See this image and copyright information in PMC

Update of

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