Format

Send to

Choose Destination
See comment in PubMed Commons below
Arch Neurol. 2012 Jun;69(6):773-7. doi: 10.1001/archneurol.2011.2441.

CD4(+) T cells predominate in cerebrospinal fluid and leptomeningeal and parenchymal infiltrates in cerebral amyloid β-related angiitis.

Author information

1
Departments of Neurology, Inflammatory Disorders of the Nervous System, and Neurooncology, University of Münster, Albert-Schweitzer-Campus 1, Münster, 48149 Germany. nico.melzer@ukmuenster.de

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.

PMID:
22351850
DOI:
10.1001/archneurol.2011.2441
[Indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments

    Supplemental Content

    Full text links

    Icon for Silverchair Information Systems
    Loading ...
    Support Center