Descriptive analysis of the Boston criteria applied to a Dutch-type cerebral amyloid angiopathy population.
OBJECTIVE
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.
van Rooden
Sanneke
S
Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands. S.van_Rooden@lumc.nl
van der Grond
Jeroen
J
van den Boom
Rivka
R
Haan
Joost
J
Linn
Jennifer
J
Greenberg
Steven M
SM
van Buchem
Mark A
MA
D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging*; D028243:Cerebral Amyloid Angiopathy, Familial / Q000000981:diagnostic imaging*; D002543:Cerebral Hemorrhage / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011859:Radiography