International Journal of Research in Medical Sciences | November 2023 | Vol 11 | Issue 11 Page 4189 International Journal of Research in Medical Sciences Luis Francisco PG et al. Int J Res Med Sci. 2023 Nov;11(11):4189-4192 www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012 Case Report Collet Sicard syndrome secondary to granulomatosis with polyangiitis Pineda Galindo Luis Francisco, Juan Josè Gòmez-Piña*, Alejandro Esteban Prado, Adriana Elizabeth Vázquez Hernández, Fernando Méndez Gallegos INTRODUCTION Granulomatosis with polyangiitis (GPA) is a systemic vasculitis that affects small and medium-sized blood vessels, with infrequent involvement of CN or hypophyseal infiltration. In 90% of patients, ANCA antibodies are positive. To induce remission, GPA is treated with immunosuppressive therapy using high-dose corticosteroids, cyclophosphamide, or rituximab. After achieving remission, patients are shifted to low-dose glucocorticoids. 1 In comparison to generalized GPA, young patients are more likely to have limited disease with a predominance in females and ANCA negativity. Limited GPA is characterized by middle ear and respiratory tract impairment, such as otitis media, sinusitis, nasal collapse, and airway stenosis. 2,3 The CSS is typically associated with malignancies, glomus jugular tumors, carotid artery dissections, trauma, and skull base osteomyelitis. However, middle ear infections can also be a less common cause of CSS. 4 Although infectious and inflammatory causes are uncommon, there have been reported cases of CSS associated with polyarteritis nodosa, Trousseau syndrome, otitis media, Lyme disease, and varicella- zoster. CSS is a rare syndrome that results in palsy of all the lower four CNs, and it is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. The jugular foramen, located at the medial end of the skull base, contains CN IX to XI, while the hypoglossal canal houses CN XII. Compression at these levels can lead to the development of CSS. 5 CASE REPORT The female of 53-year-old who complains with weight loss of 18% over the last 6 months, which is attributed to dysphagia, hearing loss, and lower CN impairment. The examination revealed several abnormalities, including Department of Internal Medicine, Hospital de Especialidades CMN La Raza, Antonio Fraga Mouret, CDMX, Mexico Received: 26 July 2023 Revised: 07 October 2023 Accepted: 09 October 2023 *Correspondence: Dr. Juan Josè Gòmez Piña, E-mail: drjgomezp@gmail.com Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Granulomatosis with polyangiitis (GPA) is a systemic vasculitis that affects small to medium-sized blood vessels. Although uncommon, cranial nerve (CN) involvement can result in CN palsy. We present a clinical case of a female with collet Sicard syndrome (CSS), displaying left-sided CN IX, X, XI, and XII involvement, which we determined to be caused by GPA. Our patient met 7 points on the American College of Rheumatology 2018 (ACR) criteria for GPA with positive ANCA, bilateral mastoiditis, and decreased sensorineural hearing. Early identification of GPA is crucial due to systemic CN involvement, as it can lead to dysphonia, dysphagia, and other complications. Early treatment can improve the functional prognosis of patients, requiring intensive induction immunosuppression due to frequent bilateral progression and worse prognosis. Despite its infrequency, timely diagnosis is critical for better patient outcomes. Keywords: Collet Sicard, Case report, CN palsy, GPA DOI: https://dx.doi.org/10.18203/2320-6012.ijrms20233398