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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Main Authors: Massimiliano Montagna, Rizvana Amir, Ilse De Volder, Martin Lammens, Jef Huyskens, Barbara Willekens
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-05-01
Series:Frontiers in Neurology
Subjects:
Online Access:http://journal.frontiersin.org/article/10.3389/fneur.2018.00329/full
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spelling doaj-61003c7bf9204d2884b5ab82d33febaa2020-11-25T01:12:45ZengFrontiers Media S.A.Frontiers in Neurology1664-22952018-05-01910.3389/fneur.2018.00329367467IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid ChangesMassimiliano Montagna0Massimiliano Montagna1Rizvana Amir2Ilse De Volder3Ilse De Volder4Martin Lammens5Jef Huyskens6Barbara Willekens7Barbara Willekens8Department of Neurology, Antwerp University Hospital, Antwerp, BelgiumMaster After Master in Medicine, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Neurology, Sint Maarten General Hospital, Mechelen, BelgiumDepartment of Neurology, Antwerp University Hospital, Antwerp, BelgiumMultidisciplinary Sleep Disorders Centre, Antwerp University Hospital, Antwerp, BelgiumDepartment of Pathology, Antwerp University Hospital, University of Antwerp, Antwerp, BelgiumDepartment of Radiology, Antwerp University Hospital, Antwerp, BelgiumDepartment of Neurology, Antwerp University Hospital, Antwerp, BelgiumLaboratory of Experimental Hematology, Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumIgLON5-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.http://journal.frontiersin.org/article/10.3389/fneur.2018.00329/fullbrain inflammationIgLON5autoimmune encephalitisrapidly evolving dementiaakathisiadyskinesia
collection DOAJ
language English
format Article
sources DOAJ
author Massimiliano Montagna
Massimiliano Montagna
Rizvana Amir
Ilse De Volder
Ilse De Volder
Martin Lammens
Jef Huyskens
Barbara Willekens
Barbara Willekens
spellingShingle Massimiliano Montagna
Massimiliano Montagna
Rizvana Amir
Ilse De Volder
Ilse De Volder
Martin Lammens
Jef Huyskens
Barbara Willekens
Barbara Willekens
IgLON5-Associated Encephalitis With Atypical Brain Magnetic Resonance Imaging and Cerebrospinal Fluid Changes
Frontiers in Neurology
brain inflammation
IgLON5
autoimmune encephalitis
rapidly evolving dementia
akathisia
dyskinesia
author_facet Massimiliano Montagna
Massimiliano Montagna
Rizvana Amir
Ilse De Volder
Ilse De Volder
Martin Lammens
Jef Huyskens
Barbara Willekens
Barbara Willekens
author_sort Massimiliano Montagna
title 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_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_sort iglon5-associated encephalitis with atypical brain magnetic resonance imaging and cerebrospinal fluid changes
publisher Frontiers Media S.A.
series Frontiers in Neurology
issn 1664-2295
publishDate 2018-05-01
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.
topic brain inflammation
IgLON5
autoimmune encephalitis
rapidly evolving dementia
akathisia
dyskinesia
url http://journal.frontiersin.org/article/10.3389/fneur.2018.00329/full
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