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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Vilnius University Press
2019-06-01
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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 |
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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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format | Article |
id | doaj-art-04afe46b809f4aeb9f352836a785f2e4 |
institution | Kabale University |
issn | 1392-3064 2424-5917 |
language | English |
publishDate | 2019-06-01 |
publisher | Vilnius University Press |
record_format | Article |
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 |
work_keys_str_mv | AT grutkauskaite multiplecranialneuropathiesinchurgstrausssyndromecasereport AT rmasaitiene multiplecranialneuropathiesinchurgstrausssyndromecasereport AT jdadoniene multiplecranialneuropathiesinchurgstrausssyndromecasereport AT djatuzis multiplecranialneuropathiesinchurgstrausssyndromecasereport |