NeurovascularMedicine.com
The contents are under continuing development and improvements and may contain errors of omission or fact. This is not to be used for the assessment, diagnosis or management of patients. It should not be regarded as medical advice by healthcare workers or laypeople. It is for educational purposes only. Please adhere to your local protocols. If you are unwell please seek urgent healthcare advice. If you do not accept this then please do not use the website.

A

Cogan syndrome


Learning objectives

  • What is Cogan syndrome
  • Recognition and treatment

Introduction

  • Cogan syndrome is a chronic inflammatory disease of unknown origin
  • Can affect young adults
  • May cause red eye, ear pain ,vertigo, episcleritis, scleritis, Uveitis, vitritis and choroiditis

Aetiology

  • Autoimmune reaction against an unknown common autoantigen in the cornea and inner ear
  • Patients can develop an aortitis.
  • Keratitis, CNS vasculitis and Deafness and venous sinus thrombosis

Clinical

  • Systemic symptoms - fever, headache, joint pain, and myalgia.
  • Bilateral sensorineural hearing loss
  • Vestibular symptoms
  • Inflammatory ocular manifestations - keratitis
  • Rarely a severe systemic vasculitis with aortitis.

Investigations

  • Bloods: mildly elevated ESR, CRP
  • Slit lamp examination examination of the eye
  • MRI and MRA brain

Differentials

  • Susac's syndrome. Though examination should readily distinguish the two disorders, the physician should consider Susac's syndrome and look for the triad of hearing loss, BRAO, encephalopathy.

Management

  • Consider ophthalmology review and systemic corticosteroids for ocular involvement
  • Systemic disease - Prednisolone 1 mg/kg po once/day is begun as soon as possible and continued for 2 to 6 mo. Some clinicians add cyclophosphamide, methotrexate, or cyclosporine for recalcitrant cases.

Note: The plan is to keep the website free through donations and advertisers that do not present any conflicts of interest. I am keen to advertise courses and conferences. If you have found the site useful or have any constructive comments please write to me at drokane (at) gmail.com. I keep a list of patrons to whom I am indebted who have contributed. If you would like to advertise a course or conference then please contact me directly for costs and to discuss a sponsored link from this site.

free web counter Hits