Sensitivity of the Boston criteria version 2.0 in Dutch-type hereditary cerebral amyloid angiopathy

Background and aim: The revised Boston criteria v2.0 for cerebral amyloid angiopathy (CAA) add two radiological markers to the existing criteria: severe visible perivascular spaces in the centrum semiovale and white matter hyperintensities (WMHs) in a multispot pattern. This study aims to determine...

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Published in:	International journal of stroke Vol. 19; no. 8; pp. 942 - 946
Main Authors:	van der Zwet, RGJ, Koemans, EA, Voigt, S, van Dort, R, Rasing, I, Kaushik, K, van Harten, TW, Schipper, MR, Terwindt, GM, van Osch, MJP, van Walderveen, MAA, van Etten, ES, Wermer, MJH
Format:	Journal Article
Language:	English
Published:	London, England SAGE Publications 01-10-2024
Subjects:	Adult Cerebral Amyloid Angiopathy, Familial - diagnosis Cerebral Amyloid Angiopathy, Familial - diagnostic imaging Cerebral Amyloid Angiopathy, Familial - genetics Cerebral Amyloid Angiopathy, Familial - pathology Cross-Sectional Studies Female Humans Magnetic Resonance Imaging - methods Male Middle Aged Mutation - genetics Netherlands Prospective Studies Sensitivity and Specificity White Matter - diagnostic imaging White Matter - pathology Netherlands cerebral amyloid angiopathy small vessel disease Intracerebral hemorrhage
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Summary:	Background and aim: The revised Boston criteria v2.0 for cerebral amyloid angiopathy (CAA) add two radiological markers to the existing criteria: severe visible perivascular spaces in the centrum semiovale and white matter hyperintensities (WMHs) in a multispot pattern. This study aims to determine the sensitivity of the updated criteria in mutation carriers with Dutch-type hereditary CAA (D-CAA) in an early and later disease stage. Methods: In this cross-sectional study, we included presymptomatic and symptomatic D-CAA mutation carriers from our prospective natural history study (AURORA) at the Leiden University Medical Center between 2018 and 2021. 3-Tesla scans were assessed for CAA-related magnetic resonance imaging (MRI) markers. We compared the sensitivity of the Boston criteria v2.0 to the previously used modified Boston criteria v1.5. Results: We included 64 D-CAA mutation carriers (mean age 49 years, 55% women, 55% presymptomatic). At least one white matter (WM) feature was seen in 55/64 mutation carriers (86%: 74% presymptomatic, 100% symptomatic). Fifteen (23%) mutation carriers, all presymptomatic, showed only WM features and no hemorrhagic markers. The sensitivity for probable CAA was similar between the new and the previous criteria: 11/35 (31%) in presymptomatic mutation carriers and 29/29 (100%) in symptomatic mutation carriers. The sensitivity for possible CAA in presymptomatic mutation carriers increased from 0/35 (0%) to 15/35 (43%) with the new criteria. Conclusion: The Boston criteria v2.0 increase the sensitivity for detecting possible CAA in presymptomatic D-CAA mutation carriers and, therefore, improve the detection of the early phase of CAA.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	1747-4930 1747-4949 1747-4949
DOI:	10.1177/17474930241239801