Skip Navigation


Brain Advance Access originally published online on January 19, 2005
Brain 2005 128(3):500-515; doi:10.1093/brain/awh379
This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow All Versions of this Article:
128/3/500    most recent
awh379v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (47)
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Scolding, N. J.
Right arrow Articles by Love, S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Scolding, N. J.
Right arrow Articles by Love, S.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

© The Author (2005). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please e-mail: journal.permissions{at}oupjournals.org

Aß-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy

Neil J. Scolding1, Fady Joseph1, Patricia A. Kirby9, Ingrid Mazanti3, Françoise Gray10, Jacqueline Mikol10, David Ellison4, David A. Hilton6, Timothy L. Williams5, James M. MacKenzie7, John H. Xuereb8 and Seth Love2

Departments of 1 Neurology and 2 Neuropathology, Institute of Clinical Neurosciences, University of Bristol, Frenchay Hospital, Bristol, 3 Department of Cellular Pathology, Division of Neuropathology, Southampton General Hospital, Southampton, 4 Department of Neuropathology, Newcastle General Hospital, 5 Department of Neurology, Royal Victoria Infirmary, Newcastle-upon-Tyne, 6 Department of Histopathology, Derriford Hospital, Plymouth, 7 Department of Pathology, Aberdeen Royal Infirmary, Aberdeen, 8 Division of Molecular Histopathology, Addenbrooke's NHS Trust, Cambridge, UK, 9 Department of Pathology, University of Iowa College of Medicine, Iowa City, IA, USA and 10 Service d'Anatomie et Cytologie Pathologiques, Hôpital Lariboisière, Paris, France

Correspondence: Dr Seth Love, Department of Neuropathology, Institute of Clinical Neurosciences, Frenchay Hospital, Bristol BS16 1LE E-mail: seth.love{at}bris.ac.uk

Idiopathic or primary angiitis of the CNS (PACNS) and cerebral amyloid angiopathy (CAA) are unusual vasculopathies generally regarded as unrelated disorders. A few case reports have, however, described granulomatous angiitis in patients with sporadic, amyloid ß peptide (Aß)-related CAA. Here we describe the clinical, neuroradiological and neuropathological features of nine patients with Aß-related angiitis (ABRA). Combining these with the individual case reports drawn from the literature has allowed us to define ABRA as a clinical entity and to compare its features with those of PACNS. The mean age of presentation of ABRA (67 years) is higher than that of PACNS but lower than that of sporadic non-inflammatory Aß-related CAA. Alterations in mental status (59%), headaches (35%), seizures and focal neurological deficits (24%) are common. Hallucinations are a presenting manifestation in 12% of cases. Most patients have white matter hyperintensities on MRI but these are of similar appearance to those in PACNS. Cerebrospinal fluid usually shows modest elevation of protein and pleocytosis. Neuropathology reveals angiodestructive inflammation, often granulomatous, and meningeal lymphocytosis. Aß is consistently present in abundance in affected blood vessels but usually scanty within the parenchyma of the cerebral cortex. However, the cortex includes numerous activated microglia, occasionally in a plaque-like distribution and containing cytoplasmic Aß. The cerebral white matter shows patchy gliosis and rarefaction, in some cases marked. Our findings (i) help to dissect one separate clinicopathological entity from what is likely to be a spectrum of primary angiitides of the CNS; (ii) have important therapeutic implications for one category of patients with amyloid-related vasculopathy; and (iii) may provide valuable insights into the development of amyloid-associated inflammation, of relevance not only to ABRA but also to Aß-immunization-related encephalitis and to Alzheimer's disease.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 NeurologyHome page
H. J. Tschampa, P. Niehusmann, M. Marek, C. -A. Mueller, K. Kuchelmeister, and H. Urbach
MRI in amyloid {beta}-related brain angiitis
Neurology, July 21, 2009; 73(3): 247 - 247.
[Full Text] [PDF]

Home page
 Arch NeurolHome page
J. Birnbaum and D. B. Hellmann
Primary Angiitis of the Central Nervous System
Arch Neurol, June 1, 2009; 66(6): 704 - 709.
[Abstract] [Full Text] [PDF]

Home page
 BMJ Case ReportsHome page
R B. Sommerville, J. M Noble, J. P. Vonsattel, R. Delapaz, and C. B Wright
Eosinophilic vasculitis in an isolated central nervous system distribution
BMJ Case Reports, February 2, 2009; 2009(jan27_1): bcr0820080832 - bcr0820080832.
[Abstract] [Full Text]

Home page
 BrainHome page
D. Boche, E. Zotova, R. O. Weller, S. Love, J. W. Neal, R. M. Pickering, D. Wilkinson, C. Holmes, and J. A. R. Nicoll
Consequence of A{beta} immunization on the vasculature of human Alzheimer's disease brain
Brain, December 1, 2008; 131(12): 3299 - 3310.
[Abstract] [Full Text] [PDF]

Home page
 Ann Rheum DisHome page
E S Molloy, A B Singhal, and L H Calabrese
Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system
Ann Rheum Dis, December 1, 2008; 67(12): 1732 - 1735.
[Abstract] [Full Text] [PDF]

Home page
 Rheumatology (Oxford)Home page
C. Salvarani, R. D. Brown Jr, K. T. Calamia, T. J. H. Christianson, J. Huston III, J. F. Meschia, C. Giannini, D. V. Miller, and G. G. Hunder
Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy
Rheumatology, November 1, 2008; 47(11): 1671 - 1677.
[Abstract] [Full Text] [PDF]

Home page
 DNA ResHome page
T. Tougan, H. Onda, D. Okuzaki, S. Kobayashi, H. Hashimoto, and H. Nojima
Focused Microarray Analysis of Peripheral Mononuclear Blood Cells from Churg-Strauss Syndrome Patients
DNA Res, April 1, 2008; 15(2): 103 - 114.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
C. Kinnecom, M. H. Lev, L. Wendell, E. E. Smith, J. Rosand, M. P. Frosch, and S. M. Greenberg
Course of cerebral amyloid angiopathy-related inflammation
Neurology, April 24, 2007; 68(17): 1411 - 1416.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
S. M. Greenberg, J. E. Parisi, and B. M. Keegan
A 63-year-old man with headaches and behavioral deterioration
Neurology, March 6, 2007; 68(10): 782 - 787.
[Full Text] [PDF]

Home page
 J. Neurosci.Home page
C. M. Prada, M. Garcia-Alloza, R. A. Betensky, S. X. Zhang-Nunes, S. M. Greenberg, B. J. Bacskai, and M. P. Frosch
Antibody-Mediated Clearance of Amyloid-{beta} Peptide from Cerebral Amyloid Angiopathy Revealed by Quantitative In Vivo Imaging
J. Neurosci., February 21, 2007; 27(8): 1973 - 1980.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
J. D. Dowell, J. D. Fleck, S. T. Vakili, and M. D. Benson
Familial oculoleptomeningeal amyloidosis associated with primary angiitis of the CNS
Neurology, January 2, 2007; 68(1): 77 - 78.
[Full Text] [PDF]

Home page
 J. Neurol. Neurosurg. PsychiatryHome page
R B Sommerville, J M Noble, J P Vonsattel, R Delapaz, and C B Wright
Eosinophilic vasculitis in an isolated central nervous system distribution
J. Neurol. Neurosurg. Psychiatry, January 1, 2007; 78(1): 85 - 88.
[Abstract] [Full Text] [PDF]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.