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Chapter 1.2 - Chapter

from 1 - Inflammatory Conditions

Published online by Cambridge University Press:  06 October 2022

Anita Arsovska
Affiliation:
University of Ss Cyril and Methodius
Derya Uluduz
Affiliation:
Istanbul Üniversitesi
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Summary

Cogan syndrome (CS) is a rare multisystem inflammatory condition characterized by ocular manifestations, classically presenting as nonsyphilitic interstitial keratitis, and audio-vestibular symptoms similar to those of Meniere’s disease. The disease evolves subacutely, with the patient typically being a young Caucasian, with no known sex predominance. Pediatric CS has also been described. The aetiology and pathogenesis of CS remain unknown, although vasculitic, infectious and autoimmune mechanisms have been proposed. The clinical signs and symptoms typically manifest as ocular and inner ear disease. Systemic manifestations present in 30%-50% of the cases, with headache, arthralgia, fever, arthritis, myalgia as the most common constitutional symptoms. Vasculitis and aortitis can also occur. Concerning neurological manifestations, the patients can present with central (stroke/ischemic events, cerebral venous thrombosis, meningitis/encephalitis) as well as peripheral nervous system (peripheral neuropathy) symptoms. Diagnosis of CS is clinical. It relies upon the presence of characteristic inflammatory eye disease and vestibulo-auditory dysfunction, with alternative causes of infection and inflammation being ruled out. The most common laboratory findings are elevated ESR and CRP, anaemia and thrombocytosis. Differential diagnosis includes sensorineural hearing loss, congenital syphilis, polyarteritis nodosa, Wegener's granulomatosis, Vogt-Koyanagi-Harada syndrome and Susac syndrome.For treatment of ocular and vestibular dysfunction, topical and systemic glucocorticoids can be used. Prognosis of CS is variable, with most patients experiencing multiple disease relapses

Type
Chapter
Information
Rare Causes of Stroke
A Handbook
, pp. 60 - 65
Publisher: Cambridge University Press
Print publication year: 2022

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References

Cogan, DG. Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol. 1945;33: 144149.CrossRefGoogle Scholar
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Study Group for Cogan’s Syndrome, Grasland, A, Pouchot, J, Hachulla, E, et al. Typical and atypical Cogan’s syndrome: 32 cases and review of the literature.Rheumatology (Oxford). 2004;438: 10071015.Google Scholar

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  • Chapter
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book: Rare Causes of Stroke
  • Online publication: 06 October 2022
  • Chapter DOI: https://doi.org/10.1017/9781108902793.012
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  • Chapter
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book: Rare Causes of Stroke
  • Online publication: 06 October 2022
  • Chapter DOI: https://doi.org/10.1017/9781108902793.012
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • Chapter
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book: Rare Causes of Stroke
  • Online publication: 06 October 2022
  • Chapter DOI: https://doi.org/10.1017/9781108902793.012
Available formats
×