Cogan's syndrome is a rare autoimmune multisystem disease. The main clinical features of typical Cogan's syndrome are vestibuloauditory dysfunction and interstitial keratitis. The authors present a case of atypical Cogan's syndrome with headache, fever, deafness, trigeminal neuralgia and electroencephalographic abnormality which mimicked viral encephalitis.
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Department of Otorhinolaryngology and Head and Neck Surgery, SLK-Kliniken Heilbronn GmbH, Heilbronn, Germany
We present a case series consisting of three female patients in their 30s with presumed autoimmune uveitis resembling Cogan's syndrome following caesarean sections (C-sections) with severe intraoperative bleeding and the use of chitosan-tamponade, exhibiting a combination of varying ocular and auditory symptoms postoperatively. Our patients displayed a range of inflammatory ocular changes, including stromal keratitis, panuveitis, retinal infiltrates, haemorrhages, optic disc swelling, and intraretinal and subretinal fluid, along with otalgia and hearing loss, consistent with typical and atypical Cogan's syndrome. Treatment involved systemic corticosteroids, resulting in variable outcomes.
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Case Report: Case report of a 51 year old patient diagnosed with atypical Cogan's syndrome. The patient exhibited interstitial keratitis, anterior uveitis, and long-standing profound deafness. The treatment was based on topical and systemic steroids, resulting in a satisfactory evolution and currently in clinical remission.
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Background: Cogan syndrome is a rare autoimmune systemic vasculitis presenting with interstitial keratitis and audiovestibular symptoms. The atypical form, characterized by more extensive ocular lesions with audiovestibular symptoms appearing with a longer delay and more frequent systemic features, is usually underdiagnosed, delaying treatment.
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Article Synopsis
- Around 10% of the U.S. population suffers from a rare disease, but patients often struggle to find knowledgeable healthcare providers and reliable information.
- A study focused on Ehlers-Danlos syndrome analyzed social interactions in support groups, primarily through Facebook, revealing that 102 respondents formed 448 connections and engaged in daily discussions.
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