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Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature Review

Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare rheumatic immune disease characterized by vasculitis of small- and medium-sized blood vessels. Central nervous system (CNS) involvement frequently consists of cerebrovascular disease; a manifestation with multiple demyelinatin...

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Autores principales: Xu, Dingkang, Xu, Hongen, Wang, Fang, Wang, Guoqing, Wei, Qingjie, Lei, Shixiong, Gao, Qiang, Zhang, Qi, Guo, Fuyou
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6423896/
https://www.ncbi.nlm.nih.gov/pubmed/30930835
http://dx.doi.org/10.3389/fneur.2019.00213
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author Xu, Dingkang
Xu, Hongen
Wang, Fang
Wang, Guoqing
Wei, Qingjie
Lei, Shixiong
Gao, Qiang
Zhang, Qi
Guo, Fuyou
author_facet Xu, Dingkang
Xu, Hongen
Wang, Fang
Wang, Guoqing
Wei, Qingjie
Lei, Shixiong
Gao, Qiang
Zhang, Qi
Guo, Fuyou
author_sort Xu, Dingkang
collection PubMed
description Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare rheumatic immune disease characterized by vasculitis of small- and medium-sized blood vessels. Central nervous system (CNS) involvement frequently consists of cerebrovascular disease; a manifestation with multiple demyelinating lesions has never been reported in detail. This report describes a 38-year-old man, who presented with progressive memory deterioration and underwent microsurgery; EGPA was subsequently confirmed. Unique clinical and radiological features as well as immunohistological outcomes and DNA sequencing revealed a potential disease-associated human leukocyte antigen (HLA) type, and single-nucleotide polymorphisms (SNPs) are described for this uncommon case. Although EGPA rarely involves the CNS, this differential diagnosis should be considered when patients present with a history of nasosinusitis, elevated eosinophil percentage, clinical pulmonitis, and neurological manifestations. Microsurgery is necessary for precise diagnosis and effective treatment.
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spelling pubmed-64238962019-03-29 Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature Review Xu, Dingkang Xu, Hongen Wang, Fang Wang, Guoqing Wei, Qingjie Lei, Shixiong Gao, Qiang Zhang, Qi Guo, Fuyou Front Neurol Neurology Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare rheumatic immune disease characterized by vasculitis of small- and medium-sized blood vessels. Central nervous system (CNS) involvement frequently consists of cerebrovascular disease; a manifestation with multiple demyelinating lesions has never been reported in detail. This report describes a 38-year-old man, who presented with progressive memory deterioration and underwent microsurgery; EGPA was subsequently confirmed. Unique clinical and radiological features as well as immunohistological outcomes and DNA sequencing revealed a potential disease-associated human leukocyte antigen (HLA) type, and single-nucleotide polymorphisms (SNPs) are described for this uncommon case. Although EGPA rarely involves the CNS, this differential diagnosis should be considered when patients present with a history of nasosinusitis, elevated eosinophil percentage, clinical pulmonitis, and neurological manifestations. Microsurgery is necessary for precise diagnosis and effective treatment. Frontiers Media S.A. 2019-03-11 /pmc/articles/PMC6423896/ /pubmed/30930835 http://dx.doi.org/10.3389/fneur.2019.00213 Text en Copyright © 2019 Xu, Xu, Wang, Wang, Wei, Lei, Gao, Zhang and Guo. http://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(s) 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 Neurology
Xu, Dingkang
Xu, Hongen
Wang, Fang
Wang, Guoqing
Wei, Qingjie
Lei, Shixiong
Gao, Qiang
Zhang, Qi
Guo, Fuyou
Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature Review
title Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature Review
title_full Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature Review
title_fullStr Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature Review
title_full_unstemmed Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature Review
title_short Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature Review
title_sort multiple distinctive demyelinating lesions caused by eosinophilic granulomatosis with polyangiitis: case report and literature review
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6423896/
https://www.ncbi.nlm.nih.gov/pubmed/30930835
http://dx.doi.org/10.3389/fneur.2019.00213
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