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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

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