Multiple cranial neuropathies in Churg-Strauss syndrome: case report

Churg-Strauss syndrome (CSS), known as eosinophilic granulomatosis with polyangiitis, is a systemic small- to medium-sized vasculitis, characterized by chronic rhinosinusitis, asthma and eosinophilia. Churg and Strauss first described this syndrome in 1951. Histology of CSS usually discloses eosino...

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Main Authors: G. Rutkauskaitė, R. Masaitienė, J. Dadonienė, D. Jatužis
Format: Article
Language:English
Published: Vilnius University Press 2019-06-01
Series:Neurologijos seminarai
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Online Access:https://www.journals.vu.lt/neurologijos_seminarai/article/view/27793
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author G. Rutkauskaitė
R. Masaitienė
J. Dadonienė
D. Jatužis
author_facet G. Rutkauskaitė
R. Masaitienė
J. Dadonienė
D. Jatužis
author_sort G. Rutkauskaitė
collection DOAJ
description Churg-Strauss syndrome (CSS), known as eosinophilic granulomatosis with polyangiitis, is a systemic small- to medium-sized vasculitis, characterized by chronic rhinosinusitis, asthma and eosinophilia. Churg and Strauss first described this syndrome in 1951. Histology of CSS usually discloses eosinophil-rich infiltrates, necrotising vasculitis, and eosinophilic-granulomatous inflammation. There are no clear pathophysiologic mechanisms that explain the development of CSS but association between CSS and HLA-DRB4 has been reported. Because of the frequent positivity of antineutrophil cytoplasmic antibodies (ANCAs), which are found in 30–40% of the cases, CSS has been grouped with ANCA-associated vasculitis. Nervous system manifestations are found in up to 70% of CSS patients, while cranial neuropathies are rarely diagnosed. Patients are treated with glucocorticoids and immunosuppressants. We describe a case report of a 68-year-old woman with a clinical diagnosis of Churg-Strauss syndrome and multiple cranial neuropathies.
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series Neurologijos seminarai
spelling doaj-art-04afe46b809f4aeb9f352836a785f2e42025-01-20T18:22:59ZengVilnius University PressNeurologijos seminarai1392-30642424-59172019-06-01232(80)10.29014/ns.2019.14Multiple cranial neuropathies in Churg-Strauss syndrome: case reportG. Rutkauskaitė 0R. Masaitienė 1J. Dadonienė 2D. Jatužis 3Vilnius University, LithuaniaVilnius University, LithuaniaVilnius University, LithuaniaVilnius University, Lithuania Churg-Strauss syndrome (CSS), known as eosinophilic granulomatosis with polyangiitis, is a systemic small- to medium-sized vasculitis, characterized by chronic rhinosinusitis, asthma and eosinophilia. Churg and Strauss first described this syndrome in 1951. Histology of CSS usually discloses eosinophil-rich infiltrates, necrotising vasculitis, and eosinophilic-granulomatous inflammation. There are no clear pathophysiologic mechanisms that explain the development of CSS but association between CSS and HLA-DRB4 has been reported. Because of the frequent positivity of antineutrophil cytoplasmic antibodies (ANCAs), which are found in 30–40% of the cases, CSS has been grouped with ANCA-associated vasculitis. Nervous system manifestations are found in up to 70% of CSS patients, while cranial neuropathies are rarely diagnosed. Patients are treated with glucocorticoids and immunosuppressants. We describe a case report of a 68-year-old woman with a clinical diagnosis of Churg-Strauss syndrome and multiple cranial neuropathies. https://www.journals.vu.lt/neurologijos_seminarai/article/view/27793Churg-Strauss syndromeeosinophilic granulomatosis with polyangiitisANCA-associated vasculitismultiple cranial neuropathiespolyneuropathy
spellingShingle G. Rutkauskaitė
R. Masaitienė
J. Dadonienė
D. Jatužis
Multiple cranial neuropathies in Churg-Strauss syndrome: case report
Neurologijos seminarai
Churg-Strauss syndrome
eosinophilic granulomatosis with polyangiitis
ANCA-associated vasculitis
multiple cranial neuropathies
polyneuropathy
title Multiple cranial neuropathies in Churg-Strauss syndrome: case report
title_full Multiple cranial neuropathies in Churg-Strauss syndrome: case report
title_fullStr Multiple cranial neuropathies in Churg-Strauss syndrome: case report
title_full_unstemmed Multiple cranial neuropathies in Churg-Strauss syndrome: case report
title_short Multiple cranial neuropathies in Churg-Strauss syndrome: case report
title_sort multiple cranial neuropathies in churg strauss syndrome case report
topic Churg-Strauss syndrome
eosinophilic granulomatosis with polyangiitis
ANCA-associated vasculitis
multiple cranial neuropathies
polyneuropathy
url https://www.journals.vu.lt/neurologijos_seminarai/article/view/27793
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AT rmasaitiene multiplecranialneuropathiesinchurgstrausssyndromecasereport
AT jdadoniene multiplecranialneuropathiesinchurgstrausssyndromecasereport
AT djatuzis multiplecranialneuropathiesinchurgstrausssyndromecasereport