Descriptive Analysis of the Boston Criteria Applied to a Dutch-Type Cerebral Amyloid Angiopathy Population
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Background and Purpose— Validation of the Boston criteria for the in vivo diagnosis of cerebral amyloid angiopathy (CAA) is challenging, because noninvasive diagnostic tests do not exist. Hereditary cerebral hemorrhage with amyloidosis–Dutch type is an accepted monogenetic model of CAA and diagnosis can be made with certainty based on DNA analysis. The aim of this study was to analyze and refine the existing Boston criteria in patients with hereditary cerebral hemorrhage with amyloidosis–Dutch type. Methods— We performed T2*-weighted MRI in 27 patients with hereditary cerebral hemorrhage with amyloidosis–Dutch type to assess the presence and location of microbleeds, intracranial hemorrhages, and superficial siderosis. Using the Boston criteria, subjects were categorized as having: no hemorrhages, possible CAA, probable CAA, and hemorrhagic lesions not qualifying for CAA. The sensitivity of the Boston criteria was calculated separately using intracranial hemorrhages only and using intracranial hemorrhages and microbleeds. Results— The sensitivity of the Boston criteria for probable CAA increased from 48% to 63% when microbleeds were included. For symptomatic subjects only, the sensitivity was 100%. No hemorrhages were identified in the deep white matter, basal ganglia, thalamus, or brainstem. Superficial siderosis, observed in 6 patients, did not increase the sensitivity of the Boston criteria in our study group. Conclusions— Our data show that using T2*-weighted MRI and including microbleeds increase the sensitivity of the Boston criteria. The exclusion of hemorrhages in the deep white matter, basal ganglia, thalamus, and brainstem does not lower the sensitivity of the Boston criteria.
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Multiple strictly lobar hemorrhages without other definite causes have been defined by the Boston criteria as probable cerebral amyloid angiopathy and validated against neuropathologically or genetically diagnosed cerebral amyloid angiopathy.
“The presence of multiple strictly lobar hemorrhages in the absence of other definite causes such as head trauma, brain tumor, or supratherapeutic anticoagulation has been defined by the Boston criteria as “probable CAA” and validated against neuropathologically or genetically diagnosed CAA.153,154”
From Vascular Contributions to Cognitive Impairment and Dementia: A Statement for Healthcare Professionals From the American Heart Association/American Stroke Association (2011) · cites this paper as [154]
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- Authors
- van Rooden, S., van der Grond, J., van den Boom, R., Haan, J., Linn, J., Greenberg, S. M., & van Buchem, M. A.
- Affiliations
- From the Departments of Radiology (S.v.R., J.v.d.G., R.v.d.B., M.A.v.B.) and Neurology (J.H.), Leiden University Medical Center, Leiden, The Netherlands; the Department of Neurology (J.H.), Rijnland Hospital, Leiderdorp, The Netherlands; the Department of Neuroradiology (J.L.), University Hospital Munich, Munich, Germany; and the Hemorrhagic Stroke Research Program (S.M.G.), Department of Neurology, Massachusetts General Hospital, Boston, Mass.
- Leiden University Medical Center (NL)
- Venue
- Stroke
- Published
- 2009-09
- Volume
- 40
- Issue
- 9
- Pages
- 3022-3027
- ISSN
- 0039-2499 (ISSN-L)
- 1524-4628
- OpenAlex
- W2151445100
- Cited by
- 129
- References
- 29
- Citations by year
- 2026: 3 · 2025: 5 · 2024: 2 · 2023: 5 · 2022: 5 · 2021: 9 · 2020: 7 · 2019: 5 · 2018: 12 · 2017: 7 · 2016: 7 · 2015: 14 · 2014: 11 · 2013: 12 · 2012: 3
- FWCI
- 5.11
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Stroke (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Neurology › Intracerebral and Subarachnoid Hemorrhage Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
- Health Sciences › Medicine › Neurology › Cerebrovascular and genetic disorders
- Keywords
- cerebral amyloid angiopathy, Boston criteria, hereditary cerebral hemorrhage with amyloidosis-Dutch type, T2-weighted MRI, microbleeds, intracranial hemorrhage, superficial siderosis, diagnostic sensitivity, basal ganglia hemorrhage, thalamic hemorrhage, brainstem hemorrhage
- MeSH terms
- Adult, Brain, Cerebral Hemorrhage, Female, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Predictive Value of Tests, Radiography, Cerebral Amyloid Angiopathy, Familial
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