TY  - JOUR
T1  - CD4+ t cells predominate in cerebrospinal fluid and leptomeningeal and parenchymal infiltrates in cerebral amyloid β–related angiitis
AU  - Melzer N, Harder A, Gross CC, et al
Y1  - 2012/06/01
N1  - 10.1001/archneurol.2011.2441
JO  - Archives of Neurology
SP  - 773
EP  - 777
VL  - 69
IS  - 6
N2  - Background 
                  In amyloid β (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 Aβ 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.
SN  - 0003-9942
M3  - doi: 10.1001/archneurol.2011.2441
UR  - http://dx.doi.org/10.1001/archneurol.2011.2441
ER  -