Brain Advance Access originally published online on January 19, 2005
Brain 2005 128(3):500-515; doi:10.1093/brain/awh379
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Aß-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy
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.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  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, August 27, 2008; (2008) ken328v1. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 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]
|
|
|
|
 |
|
 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]
|
|
|
|
|
|
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]
|
|
|
|
 |
|
 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]
|
|
|
|
|
|
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]
|
|
|
|
 |
|
 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]
|
|