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Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patie...

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Autores principales: Yoo, Il-Han, Choi, Sang Tae, Choi, Seong-Ho, Kim, Jeong-Min, Ahn, Suk-Won
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Society for Brain and Neural Science 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5491585/
https://www.ncbi.nlm.nih.gov/pubmed/28680302
http://dx.doi.org/10.5607/en.2017.26.3.168
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author Yoo, Il-Han
Choi, Sang Tae
Choi, Seong-Ho
Kim, Jeong-Min
Ahn, Suk-Won
author_facet Yoo, Il-Han
Choi, Sang Tae
Choi, Seong-Ho
Kim, Jeong-Min
Ahn, Suk-Won
author_sort Yoo, Il-Han
collection PubMed
description Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.
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spelling pubmed-54915852017-07-05 Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis Yoo, Il-Han Choi, Sang Tae Choi, Seong-Ho Kim, Jeong-Min Ahn, Suk-Won Exp Neurobiol Case Report Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions. The Korean Society for Brain and Neural Science 2017-06 2017-06-14 /pmc/articles/PMC5491585/ /pubmed/28680302 http://dx.doi.org/10.5607/en.2017.26.3.168 Text en Copyright © Experimental Neurobiology 2017. http://creativecommons.org/licenses/by-nc/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Yoo, Il-Han
Choi, Sang Tae
Choi, Seong-Ho
Kim, Jeong-Min
Ahn, Suk-Won
Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis
title Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis
title_full Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis
title_fullStr Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis
title_full_unstemmed Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis
title_short Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis
title_sort eosinophilic granulomatosis with polyangiitis presented as acute polyneuropathy and cerebral vasculitis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5491585/
https://www.ncbi.nlm.nih.gov/pubmed/28680302
http://dx.doi.org/10.5607/en.2017.26.3.168
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