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April 27, 2010; 74 (17) Articles

Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy

J. Linn, A. Halpin, P. Demaerel, J. Ruhland, A.D. Giese, M. Dichgans, M.A. van Buchem, H. Bruckmann, S.M. Greenberg
First published April 26, 2010, DOI: https://doi.org/10.1212/WNL.0b013e3181dad605
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Abstract

Background: Cerebral amyloid angiopathy (CAA) typically presents with lobar intracerebral macrohemorrhages (ICH) or microbleeds (MBs). Several case reports also found superficial siderosis (SS) in patients with CAA. We aimed to assess the value of SS for the in vivo diagnosis of CAA, and tested whether the inclusion of SS as a criterion alters the sensitivity and specificity of the Boston criteria for CAA-related hemorrhage.

Methods: We retrospectively analyzed the T2*-weighted MRIs of 38 patients with histopathologically proven CAA and of 22 control patients with histopathologically proven non-CAA ICHs regarding the presence of ICHs, MBs, and SS. We compared the sensitivity and specificity of the classic Boston criteria to that of modified criteria, which included SS as a criterion.

Results: ICHs were present in 71% of the patients with CAA, and in all control patients. MBs were found in 47.4% of patients with CAA and in 22.7% of controls. SS was detected in 60.5% of patients with CAA, but in none of the controls. The classic criteria had a sensitivity of 89.5% for CAA-related hemorrhage, while inclusion of SS increased their sensitivity to 94.7% (not significant). On the contrary, the specificity of the Boston criteria was 81.2% both for the classic and for the modified criteria.

Conclusions: Superficial siderosis (SS) occurs with high prevalence in cerebral amyloid angiopathy (CAA) and is rare in non-CAA forms of intracerebral hemorrhages. Thus, we propose that inclusion of SS in the Boston criteria might enhance their sensitivity for CAA-related hemorrhage without loss of specificity.

Glossary

CAA=
cerebral amyloid angiopathy;
ICH=
intracerebral macrohemorrhages;
MB=
microbleed;
PDw=
proton density–weighted;
SAH=
subarachnoid hemorrhage;
SS=
superficial siderosis;
T2*w=
T2*-weighted.
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Letters: Rapid online correspondence

  • Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy
    • Hong-Liang Zhang, Department of Neurology, the First Hospital of Jilin University, Changchun, China, and Karolinska Institute, Novum, plan 5, Geriatric Lab, Huddinge, SE 141 86, Stockholm, Sweden
    • NONE
    Submitted June 25, 2010
  • Reply from the authors
    • Jennifer Linn, University Hospital Munich, Department of Neuroradiology, Marchioninistrasse 15, 81377 Munich, Germany
    • Hartmut Bruckmann (Munich, Germany; hartmut.brueckmann@med.uni-muenchen,de), Steven M. Greenberg (Boston, MA; sgreenberg@partners.org)
    Submitted June 25, 2010
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