Atypical Cogan's Syndrome

Overview

Journal Case Rep Ophthalmol Med

Publisher Wiley

Specialty Ophthalmology

Date 2013 May 22

PMID 23691387

Authors

Joao Queiros

Sofia Maia

Mariana Seca

Antonio Friande

Maria Araujo

Angelina Meireles

Affiliations

Soon will be listed here.

Abstract

Background. Cogan's syndrome is a rare clinical entity whose etiopathology is still unknown, and the treatment strategies are not clearly defined. Case. A 23-year-old male presented with symptoms of headache, peripheral facial palsy, persistent right hearing loss and bilateral papillitis. Workup excluded all infectious, granulomatous, neoplastic, and immune causes. The diagnosis of atypical Cogan's syndrome was established, and the patient was treated with systemic corticosteroids and later on with cyclophosphamide and methotrexate. There were improvement of visual symptoms and stabilisation of left hearing. Conclusion. Cogan's syndrome is a very rare disease with no specific biological tests for the diagnosis. The diagnostic exams are mostly important to exclude other etiologies. The atypical ocular and audiovestibular manifestations make the diagnosis difficult, delaying the institution of appropriate therapy which may result in profound bilateral deafness.

Citing Articles

Case Report: Atypical post-COVID Cogan's syndrome.

Mezri S, Zitouni C, Sleimi W, Bouzidi M, Sameh S F1000Res. 2025; 13:1104.

PMID: 39931322 PMC: 11809643. DOI: 10.12688/f1000research.155250.1.

References

Marcos Dolado A, Sanchez Portocarrero J, Jimenez Madridejo R, Pontes Navarro J, Garcia Urra D . [Meningoencephalitis due to Rickettsia conorii. Etiopathological, clinical and diagnostic aspects]. Neurologia. 1994; 9(2):72-5. View

Ezpeleta D, Munoz-Blanco J, Tabernero C, Gimenez-Roldan S . [Neurological complications of Mediterranean boutonneuse fever. Presentation of a case of acute encephalomeningomyelitis and review of the literature]. Neurologia. 1999; 14(1):38-42. View

Amaro M, Bacellar F, Franca A . Report of eight cases of fatal and severe Mediterranean spotted fever in Portugal. Ann N Y Acad Sci. 2003; 990:331-43. DOI: 10.1111/j.1749-6632.2003.tb07384.x. View

Grasland A, Pouchot J, Hachulla E, Bletry O, Papo T, Vinceneux P . Typical and atypical Cogan's syndrome: 32 cases and review of the literature. Rheumatology (Oxford). 2004; 43(8):1007-15. DOI: 10.1093/rheumatology/keh228. View

Gluth M, Baratz K, Matteson E, Driscoll C . Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc. 2006; 81(4):483-8. DOI: 10.4065/81.4.483. View

Norton E, COGAN D . Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms; a long-term follow-up. AMA Arch Ophthalmol. 1959; 61(5):695-7. DOI: 10.1001/archopht.1959.00940090697004. View

Khairallah M, Ladjimi A, Chakroun M, Messaoud R, Ben Yahia S, Zaouali S . Posterior segment manifestations of Rickettsia conorii infection. Ophthalmology. 2004; 111(3):529-34. DOI: 10.1016/j.ophtha.2003.04.012. View

Haynes B, Mason P, Fauci A . Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine (Baltimore). 1980; 59(6):426-41. View

Tsiachris D, Deutsch M, Vassilopoulos D, Zafiropoulou R, Archimandritis A . Sensorineural hearing loss complicating severe rickettsial diseases: report of two cases. J Infect. 2007; 56(1):74-6. DOI: 10.1016/j.jinf.2007.10.002. View

10.

Allen N, Cox C, Cobo M, Kisslo J, Jacobs M, McCallum R . Use of immunosuppressive agents in the treatment of severe ocular and vascular manifestations of Cogan's syndrome. Am J Med. 1990; 88(3):296-301. DOI: 10.1016/0002-9343(90)90157-9. View

11.

Vollertsen R, McDonald T, Younge B, Banks P, W Stanson A, Ilstrup D . Cogan's syndrome: 18 cases and a review of the literature. Mayo Clin Proc. 1986; 61(5):344-61. DOI: 10.1016/s0025-6196(12)61951-x. View

12.

Kularatne S, Gawarammana I . Validity of the Weil-Felix test in the diagnosis of acute rickettsial infections in Sri Lanka. Trans R Soc Trop Med Hyg. 2009; 103(4):423-4. DOI: 10.1016/j.trstmh.2008.11.020. View

13.

Aliaga L, Sanchez-Blazquez P, Rodriguez-Granger J, Sampedro A, Orozco M, Pastor J . Mediterranean spotted fever with encephalitis. J Med Microbiol. 2009; 58(Pt 4):521-525. DOI: 10.1099/jmm.0.004465-0. View