Amyloid-β-related angiitis: a report of 2 cases with unusual presentations

被引：13

作者：

Ng, Denise W. ^{[1
,2
,7
]}

Magaki, Shino ^{[1
,2
]}

Terashima, Kevin H. ^{[1
,2
]}

Keener, Adrienne M. ^{[2
,3
]}

Salamon, Noriko ^{[2
,4
]}

Karnezis, Stellios ^{[2
,4
]}

Macyszyn, Luke ^{[2
,5
]}

Vinters, Harry V. ^{[1
,2
,3
,6
]}

机构：

[1] Univ Calif Los Angeles, Med Ctr, Dept Pathol & Lab Med, Sect Neuropathol, Los Angeles, CA 90095 USA

[2] David Geffen Sch Med, Los Angeles, CA 90095 USA

[3] Univ Calif Los Angeles, Dept Neurol, Med Ctr, Los Angeles, CA 90095 USA

[4] Univ Calif Los Angeles, Dept Radiol Sci, Med Ctr, Los Angeles, CA 90095 USA

[5] Univ Calif Los Angeles, Dept Neurosurg, Med Ctr, Los Angeles, CA 90095 USA

[6] Univ Calif Los Angeles, Brain Res Inst, Med Ctr, Los Angeles, CA 90095 USA

[7] Univ Calgary, Cumming Sch Med, Sect Neuropathol, Dept Pathol & Lab Med, Calgary, AB T2N 1N4, Canada

来源：

HUMAN PATHOLOGY | 2017年 / 64卷

关键词：

Amyloid-beta-related angiitis; Cerebral amyloid angiopathy; Central nervous system; lmmunohistochemistry; Brain biopsy; Autopsy; ANGIOPATHY-RELATED INFLAMMATION; CEREBROSPINAL-FLUID; VASCULITIS;

D O I：

10.1016/j.humpath.2017.01.008

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

Amyloid-beta-related angiitis (ABRA) is a rare complication of cerebral amyloid angiopathy in which amyloid-beta deposition in the leptomeningeal and cortical vessels is associated with vasculitis characterized by transmural lymphohistiocytic, often granulomatous, inflammation. Patients usually present with acute to subacute cognitive dysfunction, headaches, and focal neurologic deficits. We report 2 cases of ABRA with unusual clinical presentations, including one case with fatal cerebral edema leading to herniation and Duret hemorrhages, and another associated with both lobar and deep parenchymal hemorrhages with intraventricular extension as well as hypercoagulability. Both showed extensive vascular amyloid-beta deposition associated with granulomatous angiitis and foreign body type multinucleated giant cells. One of our cases demonstrates the likely effects of ABRA on impairment of fluid regulation leading to severe cerebral edema, which is an uncommon manifestation of ABRA, and may be a result of impaired blood-brain barrier function or malfunction of the neurovascular unit. (C) 2017 Elsevier Inc. All rights reserved.

引用

页码：191 / 197

页数：7

共 16 条

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