CD4+ T Cells Predominate in Cerebrospinal Fluid and Leptomeningeal and Parenchymal Infiltrates in Cerebral Amyloid β-Related Angiitis

被引：35

作者：

Melzer, Nico ^{[3
]}

Harder, Anja ^{[4
]}

Gross, Catharina C. ^{[3
]}

Woelfer, Johannes ^{[1
]}

Stummer, Walter ^{[1
]}

Niederstadt, Thomas ^{[2
]}

Meuth, Sven G. ^{[3
]}

Marziniak, Martin ^{[3
]}

Grauer, Oliver Martin ^{[3
]}

Wiendl, Heinz ^{[3
]}

机构：

[1] Univ Hosp Munster, Dept Neurosurg, Munster, Germany

[2] Univ Hosp Munster, Dept Clin Radiol, Munster, Germany

[3] Univ Munster, Dept Neurol Inflammatory Disorders Nervous Syst &, D-48149 Munster, Germany

[4] Univ Hosp Munster, Inst Neuropathol, Munster, Germany

来源：

ARCHIVES OF NEUROLOGY | 2012年 / 69卷 / 06期

关键词：

ANGIOPATHY; INFLAMMATION;

D O I：

10.1001/archneurol.2011.2441

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Background: In amyloid beta (A beta)-related angiitis (ABRA) of the central nervous system (CNS), cerebral amyloid angiopathy occurs in association with primary vasculitis of small- and medium-sized leptomeningeal and cortical arteries. It has been suggested that ABRA is triggered by vascular deposition of A beta followed by an A beta-directed (auto) immune response. Objective: To provide a detailed description of the cellular composition of the inflammatory infiltrates in the cerebrospinal fluid (CSF) and CNS and their response to immunotherapy in a typical case of ABRA. Design: Report of a single case. Setting: Neurologic referral center. Patient: 67-year-old white woman. Main Outcome Measures: Neurologic examination, magnetic resonance imaging, lumbar puncture, flow cytometry, leptomeningeal biopsy, and histopathologic analysis. Results: In a typical case of ABRA, we demonstrate for the first time the presence of a vast majority of partially activated CD4(+) T cells in CSF and leptomeningeal and parenchymal (peri) vascular infiltrates, which were frequently found in close proximity to major histocompatibility complex (MHC) class II-expressing microglia, epithelioid macrophages, and multinucleated giant cells containing intracellular deposits of A beta. Conclusion: Our findings support the notion of adaptive A beta-directed autoimmunity as the underlying pathogenic mechanism in ABRA. Arch Neurol. 2012; 69(6): 773-777. Published online February 20, 2012. doi:10.1001/archneurol.2011.2441

引用

页码：773 / 777

页数：5

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