Open Access Case report

Corticosteroid therapy in a patient with cerebral amyloid angiopathy-related inflammation

Akio Kimura1*, Takeo Sakurai1, Nobuaki Yoshikura1, Yuichi Hayashi1, Masao Takemura2, Hitoshi Takahashi3 and Takashi Inuzuka1

Author Affiliations

1 Departments of Neurology and Geriatrics, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan

2 Informative Clinical Medicine, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan

3 Department of Pathology, Brain Research Institute, Niigata University, 1-757 Asahimachi-dori, Chuo-ku, Niigata City, Japan

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Journal of Neuroinflammation 2013, 10:39  doi:10.1186/1742-2094-10-39

Published: 16 March 2013

Abstract

We studied longitudinal changes of the levels of anti-amyloid β (anti-Aβ) antibody, amyloid β (Aβ) protein, and interleukin 8 (IL-8) in cerebrospinal fluid (CSF) of a patient with cerebral amyloid angiopathy-related inflammation (CAA-ri) in whom steroid treatment resulted in clinical improvement. The diagnosis of CAA-ri was established with brain biopsy. Levels of anti-Aβ 42 antibody, Aβ 40, Aβ 42 and IL-8 in CSF were measured in the CAA-ri patient at 23 time points in the 8-month clinical course. These CSF samples were divided into 2 groups: those obtained before (n = 12) and those after (n = 11) oral corticosteroid therapy was started. We compared these levels between CSF samples obtained before and after therapy. The mean levels of anti-Aβ 42 antibody and IL-8 were significantly higher in CSF samples of the CAA-ri patient before oral corticosteroid therapy than those after therapy. A positive correlation was noted between levels of anti-Aβ 42 antibodies and IL-8 in CSF of this patient. There were no significant differences of mean levels of Aβ 40 and Aβ 42 between CSF samples obtained before and after oral corticosteroid therapy. It was possible that the autoinflammatory process with anti-Aβ 42 antibodies and IL-8 may have been involved in the pathogenesis of CAA-ri, and that corticosteroid therapy directly affected levels of anti-Aβ 42 antibody and IL-8. In summary, CAA-ri encephalopathy is a relapsing or progressive disorder and may be treatable by adequate immunosuppressive therapy. The anti-Aβ 42 antibody in CSF is a useful biological marker for therapeutic monitoring of CAA-ri.

Keywords:
Amyloid angiopathy; Antibody; Brain; Encephalopathy; Immunology