IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes

IgLON5-associated encephalitis is a syndrome with different clinical presentations consisting of sleep dysfunction, bulbar dysfunction, chorea, and progressive supranuclear palsy-like symptoms whereas dysautonomy and cognitive decline usually appear in later stages of the disease. We report a case o...

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Autores principales: Montagna, Massimiliano, Amir, Rizvana, De Volder, Ilse, Lammens, Martin, Huyskens, Jef, Willekens, Barbara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2018
Materias:
Neuroscience
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5966542/
https://www.ncbi.nlm.nih.gov/pubmed/29867738
http://dx.doi.org/10.3389/fneur.2018.00329
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author Montagna, Massimiliano
Amir, Rizvana
De Volder, Ilse
Lammens, Martin
Huyskens, Jef
Willekens, Barbara
author_facet Montagna, Massimiliano
Amir, Rizvana
De Volder, Ilse
Lammens, Martin
Huyskens, Jef
Willekens, Barbara
author_sort Montagna, Massimiliano
collection PubMed
description IgLON5-associated encephalitis is a syndrome with different clinical presentations consisting of sleep dysfunction, bulbar dysfunction, chorea, and progressive supranuclear palsy-like symptoms whereas dysautonomy and cognitive decline usually appear in later stages of the disease. We report a case of a patient with IgLON5-associated encephalitis presenting with rapidly progressive cognitive decline and atypical inflammatory lesions on brain magnetic resonance imaging, oligoclonal bands on cerebrospinal fluid, anti-IgLON5 antibodies exclusively of the IgG1 class, and a fierce inflammatory reaction on brain biopsy, who responded favorably to immunotherapy.
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spelling pubmed-59665422018-06-04 IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes Montagna, Massimiliano Amir, Rizvana De Volder, Ilse Lammens, Martin Huyskens, Jef Willekens, Barbara Front Neurol Neuroscience IgLON5-associated encephalitis is a syndrome with different clinical presentations consisting of sleep dysfunction, bulbar dysfunction, chorea, and progressive supranuclear palsy-like symptoms whereas dysautonomy and cognitive decline usually appear in later stages of the disease. We report a case of a patient with IgLON5-associated encephalitis presenting with rapidly progressive cognitive decline and atypical inflammatory lesions on brain magnetic resonance imaging, oligoclonal bands on cerebrospinal fluid, anti-IgLON5 antibodies exclusively of the IgG1 class, and a fierce inflammatory reaction on brain biopsy, who responded favorably to immunotherapy. Frontiers Media S.A. 2018-05-17 /pmc/articles/PMC5966542/ /pubmed/29867738 http://dx.doi.org/10.3389/fneur.2018.00329 Text en Copyright © 2018 Montagna, Amir, De Volder, Lammens, Huyskens and Willekens. https://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Montagna, Massimiliano
Amir, Rizvana
De Volder, Ilse
Lammens, Martin
Huyskens, Jef
Willekens, Barbara
IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes
title IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes
title_full IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes
title_fullStr IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes
title_full_unstemmed IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes
title_short IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes
title_sort iglon5-associated encephalitis with atypical brain magnetic resonance imaging and cerebrospinal fluid changes
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5966542/
https://www.ncbi.nlm.nih.gov/pubmed/29867738
http://dx.doi.org/10.3389/fneur.2018.00329
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