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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Main Authors: Dingkang Xu, Hongen Xu, Fang Wang, Guoqing Wang, Qingjie Wei, Shixiong Lei, Qiang Gao, Qi Zhang, Fuyou Guo
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-03-01
Series:Frontiers in Neurology
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fneur.2019.00213/full
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author Dingkang Xu
Hongen Xu
Fang Wang
Guoqing Wang
Qingjie Wei
Shixiong Lei
Qiang Gao
Qi Zhang
Qi Zhang
Fuyou Guo
Fuyou Guo
author_facet Dingkang Xu
Hongen Xu
Fang Wang
Guoqing Wang
Qingjie Wei
Shixiong Lei
Qiang Gao
Qi Zhang
Qi Zhang
Fuyou Guo
Fuyou Guo
author_sort Dingkang Xu
collection DOAJ
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 doaj.art-09777d7697ae437eb46c5733b97debd22022-12-21T18:44:05ZengFrontiers Media S.A.Frontiers in Neurology1664-22952019-03-011010.3389/fneur.2019.00213439334Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature ReviewDingkang Xu0Hongen Xu1Fang Wang2Guoqing Wang3Qingjie Wei4Shixiong Lei5Qiang Gao6Qi Zhang7Qi Zhang8Fuyou Guo9Fuyou Guo10Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaCenter for Precision Medicine, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaDepartment of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaDepartment of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaDepartment of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaDepartment of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaDepartment of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaCenter for Precision Medicine, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaSchool of Pharmaceutical Sciences, Zhengzhou University, Zhengzhou, ChinaDepartment of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaKey Laboratory of Neurosurgical Diseases, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaEosinophilic 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.https://www.frontiersin.org/article/10.3389/fneur.2019.00213/fullEGPADNA sequencingHLASNPCNS involvementneurosurgery
spellingShingle Dingkang Xu
Hongen Xu
Fang Wang
Guoqing Wang
Qingjie Wei
Shixiong Lei
Qiang Gao
Qi Zhang
Qi Zhang
Fuyou Guo
Fuyou Guo
Multiple Distinctive Demyelinating Lesions Caused by Eosinophilic Granulomatosis With Polyangiitis: Case Report and Literature Review
Frontiers in Neurology
EGPA
DNA sequencing
HLA
SNP
CNS involvement
neurosurgery
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 EGPA
DNA sequencing
HLA
SNP
CNS involvement
neurosurgery
url https://www.frontiersin.org/article/10.3389/fneur.2019.00213/full
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