scholarly journals A Rare Case of Atypical Cogan’s Syndrome Presenting as Encephalitis

2021 ◽  
Author(s):  
Debashis Maikap ◽  
Amrita Pradhan ◽  
Prasanta Padhan
Keyword(s):  
Immunosuppressive Treatment ◽  
Young Patients ◽  
Confirmatory Test ◽  
High Morbidity ◽  
Unknown Aetiology ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis ◽  
Vestibular Symptoms ◽  
Systemic Symptoms

Abstract Cogan’s syndrome (CS) is a rare autoimmune vasculitis of unknown aetiology characterised by non-syphilitic interstitial keratitis, audio-vestibular symptoms, sometimes systemic symptoms and multi-organ involvement. Atypical CS has other ocular features such as scleritis, episcelritis, retinitis and optic neuritis. Diagnosis of CS is purely clinical without a confirmatory test. Hereby, we report a case of atypical CS presenting with features of encephalitis who was treated successfully with intravenous pulse methylprednisolone with cyclophosphamide. It is important to consider Cogan’s syndrome in the differential diagnosis of encephalitis with ocular and vestibular symptoms in young patients, as high morbidity and mortality rates are effectively lowered by early immunosuppressive treatment.

scholarly journals Interstitial keratitis in patients with Cogan’s Syndrome

2017 ◽  
Vol 76 (1) ◽  
Author(s):  
Thiago Gonçalves dos Santos Martins ◽  
Ana Luiza Fontes de Azevedo Costa ◽  
Thomaz Gonçalves dos Santos Martins
Keyword(s):  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis

scholarly journals Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan’s Syndrome

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Ahad Azami ◽  
Nasrollah Maleki ◽  
Mohammadreza Kalantar Hormozi ◽  
Zahra Tavosi
Keyword(s):  
Hearing Loss ◽  
Systemic Vasculitis ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Conjunctival Hyperemia ◽  
Cogan’S Syndrome ◽  
Auditory Brain Stem Response ◽  
Chronic Inflammatory Disorder ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis

Cogan’s syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan’s syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement.

Side selection for cochlear implantation in a case of Cogan's syndrome

2007 ◽  
Vol 122 (3) ◽  
pp. 310-313 ◽  
Author(s):  
G J Im ◽  
H H Jung
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Steroid Treatment ◽  
Radiological Findings ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis ◽  
Selection For ◽  
Bilateral Deafness ◽  
Selection Of

AbstractCogan's syndrome is a rare clinical entity that is characterised by non-syphilitic interstitial keratitis and audiovestibular symptoms. The cause of Cogan's syndrome is considered to be autoimmune disease, which is supported by the resolution of hearing loss after steroid treatment, and the association with other autoimmune diseases. The sensorineural hearing loss of Cogan's syndrome is progressive over a few months, and sudden, bilateral deafness often occurs, which may be an indication for cochlear implantation. This paper presents the case of a young woman suffering from Cogan's syndrome and sudden, bilateral deafness. With reference to this case, we describe problems regarding cochlear implantation for Cogan's syndrome and radiological findings aiding selection of cochlear implantation side.

scholarly journals Cogan's syndrome – A rare aortitis, difficult to diagnose but with therapeutic potential

2017 ◽  
Vol 63 (12) ◽  
pp. 1028-1031 ◽  
Author(s):  
Raiza Colodetti ◽  
Guilherme Spina ◽  
Tatiana Leal ◽  
Mucio Oliveira Jr ◽  
Alexandre Soeiro
Keyword(s):  
Disease Control ◽  
Rare Case ◽  
Aortic Wall ◽  
Therapeutic Potential ◽  
Immunosuppressive Treatment ◽  
Rare Condition ◽  
Cogan’S Syndrome ◽  
Ocular Manifestations ◽  
Cogan's Syndrome

Summary The inflammation of aortic wall, named aortitis, is a rare condition that can be caused by a number of pathologies, mainly inflammatory or infectious in nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome, making it the rare case, but susceptible to adequate immunosuppressive treatment and satisfactory disease control.

Cogan's syndrome: State of the art of systemic immunosuppressive treatment in adult and pediatric patients

2017 ◽  
Vol 16 (4) ◽  
pp. 385-390 ◽  
Author(s):  
Paolo Mora ◽  
Giacomo Calzetti ◽  
S. Ghirardini ◽  
P. Rubino ◽  
S. Gandolfi ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
State Of The Art ◽  
Immunosuppressive Treatment ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome

Corneal Autoimmunity in Cogan's Syndrome?

1992 ◽  
Vol 101 (8) ◽  
pp. 679-684 ◽  
Author(s):  
Maarten H. J. M. Majoor ◽  
Frans W. J. Albers ◽  
Frits Gmelig-Meyling ◽  
Ruth van der Gaag ◽  
Egbert H. Huizing
Keyword(s):  
Corticosteroid Therapy ◽  
Cogan’S Syndrome ◽  
Systemic Corticosteroids ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis ◽  
High Doses ◽  
Autoimmune Reactivity ◽  
Autoimmune Pathogenesis

Autoimmune reactivity against corneal antigens is described in two patients with Cogan's syndrome, a nonsyphilitic deep interstitial keratitis with vestibuloauditory symptoms. In both cases corneal antibodies were found at the beginning or during an exacerbation of the disease. After administration of high doses of corticosteroids the corneal antibodies diminished. Interstitial keratitis can generally be controlled by local or systemic corticosteroids. The effect of corticosteroid therapy on the audiovestibular symptoms is variable. The possibility of an autoimmune pathogenesis of Cogan's syndrome is discussed.

scholarly journals Autoimmune ear disease: clinical and diagnostic relevance in Cogan’s sydrome

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Luigi Maiolino ◽  
Salvatore Cocuzza ◽  
Angelo Conti ◽  
Luisa Licciardello ◽  
Agostino Serra ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Systemic Vasculitis ◽  
Clinical Syndrome ◽  
Inflammatory Disorder ◽  
Cogan’S Syndrome ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease

The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan’s syndrome. Cogan’s syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis, vestibular and auditory dysfunction. Associations between Cogan’s syndrome and systemic vasculitis, as well as aortitis, also exist. We report a case of a young woman who presented audiological and systemic characteristics attributable to Cogan’s syndrome. In the description of the case we illustrate how the appearance and evolution of the disease presented.

scholarly journals Case Report: Effective and Safe Treatment With Certolizumab Pegol in Pregnant Patients With Cogan’s Syndrome: A Report of Three Pregnancies in Two Patients

2021 ◽  
Vol 11 ◽  
Author(s):  
Nils Venhoff ◽  
Jens Thiel ◽  
Markus A. Schramm ◽  
Ilona Jandova ◽  
Reinhard E. Voll ◽  
...  
Keyword(s):  
Inflammatory Diseases ◽  
Treatment Options ◽  
Certolizumab Pegol ◽  
Immunosuppressive Agents ◽  
Immunosuppressive Treatment ◽  
Cogan’S Syndrome ◽  
Safe Treatment ◽  
Tnf Α ◽  
Cogan's Syndrome ◽  
Systemic Glucocorticoids

Cogan’s syndrome is a rare autoimmune disease characterized by ocular inflammation and audiovestibular manifestations. Treatment consists of systemic glucocorticoids and other immunosuppressive agents including methotrexate, cyclophosphamide and TNF-α-inhibitors. Due to potential ovarian or fetal toxicity immunosuppressive treatment options are limited during pregnancies. Thus far there is a paucity of reports on pregnancies in Cogan’s syndrome. With minimal transplacental transfer, Certolizumab pegol is considered to be safe for the use in pregnant patients with underlying inflammatory diseases. However, there is no literature on the use of this TNF-α-inhibitor in Cogan’s syndrome in general and especially during gestation. Here we report three pregnancies in two Cogan’s Syndrome-patients treated with Certolizumab pegol. Treatment with Certolizumab pegol was effective and well tolerated in patients with Cogan’s syndrome and seems to be a safe treatment option during pregnancy.

Sign in / Sign up

Export Citation Format

Share Document