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01-02-2019 | Original Communication

Patient characteristics and outcome associations in AMPA receptor encephalitis

Authors: Osvaldo Laurido-Soto, Matthew R. Brier, Laura E. Simon, Austin McCullough, Robert C. Bucelli, Gregory S. Day

Published in: Journal of Neurology | Issue 2/2019

Abstract

Antibody-mediated encephalitis defines a class of diseases wherein antibodies directed at cell-surface receptors are associated with behavioral and cognitive disturbances. One such recently described encephalitis is due to antibodies directed at alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptors (AMPAR). This entity is exceptionally rare and its clinical phenotype incompletely described. We present findings from two cases of AMPAR encephalitis that exemplify variability in the disease spectrum, and summarize findings in published cases derived from a systematic literature review. When all patients are considered together, the presence of psychiatric symptoms at presentation portended a poor outcome and was associated with the presence of a tumor. Furthermore, we provide evidence to suggest that the topography of magnetic resonance imaging abnormalities in reported cases mirrors the distribution of AMPARs in the human brain. The potential for neurological improvement following immunomodulatory therapy together with the favorable outcome reported in most cases emphasizes the importance of testing for autoantibodies against neuronal cell-surface proteins, including AMPAR, in patients with clinical and neuroimaging findings suggestive of autoimmune encephalitis. Close attention to the clinical phenotype may inform the presence of malignancy and long-term prognosis.

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Title: Patient characteristics and outcome associations in AMPA receptor encephalitis
Authors: Osvaldo Laurido-Soto
Matthew R. Brier
Laura E. Simon
Austin McCullough
Robert C. Bucelli
Gregory S. Day
Publication date: 01-02-2019
Publisher: Springer Berlin Heidelberg
Published in: Journal of Neurology / Issue 2/2019
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-018-9153-8

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Patient characteristics and outcome associations in AMPA receptor encephalitis

Abstract

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Springer Medicine

Abstract

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