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Amyloid β-related Angiitis of the Central Nervous System: Report of 3 Cases

Published online by Cambridge University Press:  02 December 2014

Heather Rigby ,
Alexander Easton  and
Virender Bhan
Heather Rigby*
Affiliation:
Dalhousie University, Halifax, Nova Scotia, Canada QEII Health Sciences Centre, Halifax, Nova Scotia, Canada
Alexander Easton
Affiliation:
Dalhousie University, Halifax, Nova Scotia, Canada QEII Health Sciences Centre, Halifax, Nova Scotia, Canada
Virender Bhan
Affiliation:
Dalhousie University, Halifax, Nova Scotia, Canada QEII Health Sciences Centre, Halifax, Nova Scotia, Canada
*
Dalhousie University, Division of Neurology, Halifax Infirmary, 1796 Summer St., Room 3383, Halifax, Nova Scotia, B3H 3A7, Canada
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Abstract

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Objective:

Amyloid-β (Aβ) related angiitis (ABRA) is a recently described clinicopathological entity characterized by cerebrovascular Aβ deposition and arteritis. Cerebral Aβ deposition is commonly present in cerebal amyloid angiopathy (CAA) and Alzheimer's disease (AD) but is rarely associated with inflammatory infiltration of vessel walls. Our objective is to help clarify the clinical spectrum, radiographic findings, response to treatment, and outcomes of ABRA. The neuropathological relationship between ABRA, cerebral amyloid angiopathy, and Alzheimer's disease is discussed.

Methods:

We present three cases of ABRA managed at a tertiary care centre.

Results:

All three patients presented with seizures and cognitive dysfunction; one had multifocal neurologic findings. Brain biopsies revealed inflammatory arteritis with Aβ deposits in the vessel walls. All were treated with steroids and cyclophosphamide. Two had favorable outcomes and one stabilized but with severe residual neurologic disability.

Conclusions:

ABRA is an unusual but likely under-recognized and potentially treatable disorder. As in other reported cases, our findings suggest that many patients respond favorably to immunosuppressive therapy. We believe that all biopsy specimens consistent with primary angiitis of the central nervous system (CNS) should be further examined for vascular Aβ deposition.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 38 , Issue 4 , July 2011 , pp. 626 - 630
Copyright
Copyright © The Canadian Journal of Neurological 2011

References

1.Scolding, NJ, Joseph, F, Kirby, PA, et al.Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain. 2005;128(Pt 3):500–15.Google Scholar
2.Reid, AH, Maloney, AF.Giant cell arteritis and arteriolitis associated with amyloid angiopathy in an elderly mongol. Acta Neuropathol. 1974;27(2):131–7.Google Scholar
3.Calabrese, LH, Mallek, JA.Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore). 1988;67 (1):20–39.Google Scholar
4.Alrawi, A, Trobe, JD, Blaivas, M, Musch, DC.Brain biopsy in primary angiitis of the central nervous system. Neurology. 1999;53(4):858–60.Google Scholar
5.Salvarani, C, Brown, RD Jr., Calamia, KT, et al.Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Rheumatology (Oxford). 2008;47(11):1671–7.Google Scholar
6.Eng, JA, Frosch, MP, Choi, K, et al.Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol. 2004;55(2):250–6.CrossRefGoogle ScholarPubMed
7.Chung, KK, Anderson, NE, Hutchinson, D, et al.Cerebral amyloid angiopathy related inflammation: three case reports and a review. J Neurol Neurosurg Psychiatry.82(1):20–6.Google Scholar
8.Winkler, DT, Bondolfi, L, Herzig, MC, et al.Spontaneous hemorrhagic stroke in a mouse model of cerebral amyloid angiopathy. J Neurosci. 2001;21(5):1619–27.Google Scholar
9.Kinnecom, C, Lev, MH, Wendell, L, et al.Course of cerebral amyloid angiopathy-related inflammation. Neurology. 2007;68(17): 1411–6.CrossRefGoogle ScholarPubMed
10.Lange, M, Feiden, W.Amyloid angiopathy-a rare cause of intracerebral hemorrhage. Neurosurg Rev. 1991;14(4):297–301.Google Scholar
11.Calabrese, LH, Mallek, JA.The treatment of vasculitis of the central nervous system. JAMA. 1987;258(6):778.Google Scholar
12.Newell, KL, Hyman, BT, Growdon, JH, Hedley-Whyte, ET.Application of the National Institute on Aging (NIA)-Reagan Institute criteria for the neuropathological diagnosis of Alzheimer disease. J Neuropathol Exp Neurol. 1999;58(11): 1147–55.CrossRefGoogle ScholarPubMed
13.Tschampa, HJ, Niehusmann, P, Marek, M, et al.MRI in amyloid beta-related brain angiitis. Neurology. 2009;73(3):247.CrossRefGoogle ScholarPubMed
14.Brotman, DJ, Eberhart, CG, Burger, PC, et al.Primary angiitis of the central nervous system and Alzheimer’s disease: clinically and pathologically evident in a single patient. J Rheumatol. 2000; 27(12):2935–7.Google Scholar
15.Coria, F, Rubio, I.Cerebral amyloid angiopathies. Neuropathol Appl Neurobiol. 1996;22(3):216–27.Google Scholar