The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies.

The autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow...

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Main Authors: Irani, SR, Vincent, A
Format: Journal article
Language:English
Published: 2012
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author Irani, SR
Vincent, A
author_facet Irani, SR
Vincent, A
author_sort Irani, SR
collection OXFORD
description The autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow the specificity of the underlying antibody to be confidently predicted. Antibodies against the VGKC-complex, mainly LGI1(leucine-rich glioma-inactivated 1), CASPR2 (contactin-associated protein 2), and contactin-2, and NMDA (N-methyl, D-aspartate) -receptor are the most frequently established serological associations. In the minority of cases, an underlying tumour can be responsible. Early administration of immunotherapies, and tumour removal, where it is relevant, offer the greatest chance of improvement. Prolonged courses of immunotherapies may be required, and clinical improvements often correlate well with the antibody levels. In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field.
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spelling oxford-uuid:2baf526a-f9ad-4734-ac19-83e4956c57eb2022-03-26T12:32:27ZThe expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:2baf526a-f9ad-4734-ac19-83e4956c57ebEnglishSymplectic Elements at Oxford2012Irani, SRVincent, AThe autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow the specificity of the underlying antibody to be confidently predicted. Antibodies against the VGKC-complex, mainly LGI1(leucine-rich glioma-inactivated 1), CASPR2 (contactin-associated protein 2), and contactin-2, and NMDA (N-methyl, D-aspartate) -receptor are the most frequently established serological associations. In the minority of cases, an underlying tumour can be responsible. Early administration of immunotherapies, and tumour removal, where it is relevant, offer the greatest chance of improvement. Prolonged courses of immunotherapies may be required, and clinical improvements often correlate well with the antibody levels. In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field.
spellingShingle Irani, SR
Vincent, A
The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies.
title The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies.
title_full The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies.
title_fullStr The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies.
title_full_unstemmed The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies.
title_short The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies.
title_sort expanding spectrum of clinically distinctive immunotherapy responsive autoimmune encephalopathies
work_keys_str_mv AT iranisr theexpandingspectrumofclinicallydistinctiveimmunotherapyresponsiveautoimmuneencephalopathies
AT vincenta theexpandingspectrumofclinicallydistinctiveimmunotherapyresponsiveautoimmuneencephalopathies
AT iranisr expandingspectrumofclinicallydistinctiveimmunotherapyresponsiveautoimmuneencephalopathies
AT vincenta expandingspectrumofclinicallydistinctiveimmunotherapyresponsiveautoimmuneencephalopathies