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: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Vilnius University Press
2019-06-01
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| Series: | Neurologijos seminarai |
| Subjects: | |
| Online Access: | https://www.journals.vu.lt/neurologijos_seminarai/article/view/27793 |
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| Summary: | 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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| ISSN: | 1392-3064 2424-5917 |