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  • Title: CD4(+) T cells predominate in cerebrospinal fluid and leptomeningeal and parenchymal infiltrates in cerebral amyloid β-related angiitis.
    Author: Melzer N, Harder A, Gross CC, Wölfer J, Stummer W, Niederstadt T, Meuth SG, Marziniak M, Grauer OM, Wiendl H.
    Journal: Arch Neurol; 2012 Jun; 69(6):773-7. PubMed ID: 22351850.
    Abstract:
    BACKGROUND: In amyloid (Aβ)-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 followed by an Aβ-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 Aβ. CONCLUSION: Our findings support the notion of adaptive Aß-directed autoimmunity as the underlying pathogenic mechanism in ABRA.
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