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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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 |
work_keys_str_mv |
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