Multiple cranial neuropathies in Churg-Strauss syndrome: case report
Case Reports
G. Rutkauskaitė
Vilnius University, Lithuania
R. Masaitienė
Vilnius University, Lithuania
J. Dadonienė
Vilnius University, Lithuania
D. Jatužis
Vilnius University, Lithuania
Published 2019-06-01
https://doi.org/10.29014/ns.2019.14
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Keywords

Churg-Strauss syndrome
eosinophilic granulomatosis with polyangiitis
ANCA-associated vasculitis
multiple cranial neuropathies
polyneuropathy

How to Cite

1.
Rutkauskaitė G, Masaitienė R, Dadonienė J, Jatužis D. Multiple cranial neuropathies in Churg-Strauss syndrome: case report. NS [Internet]. 2019 Jun. 1 [cited 2024 May 6];23(2(80):92-7. Available from: https://www.journals.vu.lt/neurologijos_seminarai/article/view/27793

Abstract

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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