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(Stroke. 2009;40:3022.)
© 2009 American Heart Association, Inc.

Original Contributions

Descriptive Analysis of the Boston Criteria Applied to a Dutch-Type Cerebral Amyloid Angiopathy Population

Sanneke van Rooden, MSc; Jeroen van der Grond, PhD; Rivka van den Boom, MD, PhD; Joost Haan, MD, PhD; Jennifer Linn, MD; Steven M. Greenberg, MD, PhD Mark A. van Buchem, MD, PhD

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.

Correspondence to S. van Rooden, MSc, Department of Radiology, C3-Q, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands. E-mail S.van_Rooden{at}lumc.nl

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.

Key Words: cerebral amyloid angiopathy • hemorrhage • MRI • neuroradiology