NEUROIMAGES

MRI in amyloid ␤–related brain angiitis

Figure

Edema and microbleeds as hallmark of amyloid ␤ angiitis

Fluid-attenuated inversion recovery (A), T2-weighted gradient echo (B), and high-resolution fast spin echo (C) images show
bitemporal edema (A, B: arrows) and cortical microbleeds (punctuate areas marked by arrows in B and C). Microbleeds are
present in edematous and nonedematous brain regions (D). Histopathology reveals inflammation and hyaline thickening of
leptomeningeal and cortical vessel walls in hematoxylin-eosin stain (E), Congo red–positive vascular amyloid deposition
with inflammation (E, inset), A␤ deposits (dark brown) in vessel walls, and diffuse cortical plaques (F, A␤ immunohistology).

A 52-year-old man presented with complex partial seizures. Fluid-attenuated inversion recovery MRI showed
multifocal subcortical and cortical edema, predominantly in the left temporal lobe (figure, A). T2-weighted and
gradient echo images showed multiple cortical microbleeds (figure, B–D). Right frontal brain biopsy revealed
amyloid ␤–related angiitis showing leptomeningeal and cortical blood vessels with vascular ␤–amyloid deposition
and perivascular/intramural inflammatory infiltrates with lymphocytes and often epithelioid appearing, sometimes
multinucleated macrophages (figure, E).1,2 There were also diffuse cortical ␤-amyloid plaques without deposition of
abnormal ␶-protein (figure, F). That cortical microbleeds are present not only in edematous brain, but also in
unaffected regions, likely reflects the pathophysiologic cascade of amyloid deposition followed by inflammation.
H.J. Tschampa, MD, P. Niehusmann, MD, M. Marek, MD, C.-A. Mueller, MD, K. Kuchelmeister, MD, PhD,
H. Urbach, MD, PhD, Bonn, Germany
Disclosure: Dr. Tschampa, Dr. Niehusmann, Dr. Marek, Dr. Mueller, and Dr. Kuchelmeister report no disclosures. Dr. Urbach
serves on the editorial board of Clinical Neuroradiology.
Address correspondence and reprint requests to Dr. Henriette J. Tschampa, Department of Radiology, Neuroradiology, University of Bonn,
Sigmund-Freud-Strasse 25, D-53105 Bonn, Germany; Henriette.Tschampa@ukb.uni-bonn.de
1.
2.

Scolding NJ, Joseph F, Kirby PA, et al. A␤-related angiitis: primary angiitis of the central nervous system associated with
cerebral amyloid angiopathy. Brain 2005;128:500 –515.
Marotti JD, Savitz SI, Kim WK, Williams K, Caplan LR, Joseph JT. Cerebral amyloid angiitis progressing to generalized
angiitis and leukoencephalitis. Neuropathol Appl Neurobiol 2007;33:475– 479.
Copyright © 2009 by AAN Enterprises, Inc.

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MRI in amyloid β−related brain angiitis
H. J. Tschampa, P. Niehusmann, M. Marek, et al.
Neurology 2009;73;247
DOI 10.1212/WNL.0b013e3181ae7cd0
This information is current as of July 20, 2009
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