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Results for “Cogan Syndrome”

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At least 19 recordsLinked to original sources

PubMed1978

Neurologic manifestations of Cogan syndrome.

Cogan syndrome is a multisystem inflammatory vascular disease, characterized by nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Recent reports have directed attention to involvement of other organ systems. Respiratory, cardiovascular, gastrointestinal, and musculoskeletal problems are common, as are laboratory abnormalities and general symptoms such as fever, chills, and weight loss. Prominent neurologic problems in two patients prompted a review of 79 cases of Cogan syndrome. More than half had nervous system involvement, including electroencephalographic or spinal fluid abnormality, headache, psychosis, coma, convulsion, neuropathy, and stroke. Cogan syndrome should be considered when neurologic deficits are accompanied by eye, ear, and systemic symptoms.

Adult↗

PubMed1975

[The Cogan syndrome].

Exactly 30 years ago D. C. Cogan reported 4 cases of "nonsyphilitic interstitial keratitis associated with vestibulo-auditory symptoms". Report of 57 of these cases have come to our attention. They mostly concern relatively young people, although the age range is now 4-1/2 to 63 years. The etiology of Cogan's syndrome is unknown. It very frequently has been associated with some generalized vascular diseases. In our first case, a 13 year old boy, we found the symptoms of a serous meningitis with pathological signs in the EEG. No vascular changes were encountered. During 4 weeks the rapidly progressive hearing loss was accompanied by tinnitus and vertigo and an interstitial keratitis. The recession of vestibular symptoms is followed by the loss of function of the endorgans. The voice seemed high-pitched and monotonous. In our patient the eye condition improved, but the cochleo-vestibular disturbances did not. The second case, a 27 year old woman, could be observed over a period of 19 years. The ocular symptoms disappeared nearly completely after 1 year, but the cochleo-vestibular disturbances remained. The audition shows even after 19 years some fluctuations.

Adolescent↗

PubMed1992

[Results of audiometric and vestibular tests in Cogan syndrome: apropos of a case].

The authors report on a case of Cogan's syndrome pointing out that hearing loss is due both to cochlear lesions from likely immunitary origin as well as to widespread alterations of auditory central nervous structures. This interpretation allows to explain the apparent discrepancy of the found clinical and instrumental data. From a therapeutic point of view, the authors assert the necessity of an early corticosteroid therapy to obtain some appreciable result. Anyway, they point out how prognosis for auditory function in Cogan's syndrome is often very poor.

Adult↗

PubMed1992

[Cardiovascular manifestations of Cogan syndrome. Apropos of a case].

The authors describe a case of Cogan's syndrome in a patient with ulcerative colitis complicated by several cardiovascular manifestations including bilateral coronary ostial stenosis, rapidly progressive aortic regurgitation and aneurysm of the thoracic aorta, thrombosis of the common iliac artery and pericardial symphysis. This rare form of inflammatory arteritis, the diagnosis of which is usually made on the finding of associated ocular and auditory involvement, is distinct from other types of angiitis by the predisposition to severe cardiovascular complications which influence the vital prognosis. The differential diagnosis with more common collagen diseases with cardiovascular complications is discussed.

Adult↗

PubMed1979

[Twins with congenital oculomotor apraxia (Cogan's syndrome)].

Cogan (1952) described a syndrome he called congenital ocular motor apraxia. The syndrome is characterized by 1. absence of voluntary gaze movements and fast phase of optokinetic response in horizontal direction, 2. typical jerky head movements in attempted gaze to either side, 3. nomral random movements and 4. normal vertical eye movements. A report is given on this disorder in the case of female twins.

Apraxias↗

PubMed1976

Cogan syndrome associated with mesenteric vascular insufficiency.

Nonsyphilitic interstitial keratitis and deafness was first described as a distinct entity by Cogan in 1945. For years thereafter, it was believed to involve only the ocular and aural systems. It is gradually becoming apparent that the syndrome can also include other organ systems by affecting their blood supply. A patient with diagnosed Cogan syndrome subsequently developed mesenteric vascular insufficiency that was surgically corrected. This report lends more support to the belief that nonsyphilitic interstitial keratitis with deafness is only one manifestation of a more generalized vascular disease.

Adult↗

PubMed1977

[Cogan's syndrome].

Since Cogan described his first case in 1945, about 50 cases have been published in the world literature, A 23 year old patient who suffered from this disease led us to make a detailed study of previous publications. The cochleo-vestibular, the etiological and the ophtalmical aspects of the case were studied, and, also the differential diagnosis and the therapeutical possibilities.

Adult↗

PubMedDate not supplied

Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome: a spectrum of disease.

Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome are considered to be variations of a single disease process, which is characterized by abnormalities of the cornea, anterior chamber angle, and iris. In each variation, the typical patient is a white woman with unilateral disease, negative family history, and an onset of symptoms in early to middle adulthood. Since the membrane theory of Campbell suggests that the disease is a fundamental abnormality of the corneal endothelium, rather than the iris, the term "iridocorneal endothelial syndrome," as proposed by Yanoff, may be an appropriate inclusive term for the spectrum of disease, although further study of the pathogenesis is needed. For each variation of the disease, corneal edema and secondary glucoma are both treated primarily by medical or surgical reduction of the intraocular pressure, although penetrating keratoplasty is occasionally required for cases with advanced corneal edema.

Adult↗

PubMed1992

Cogan's syndrome: auditory and medical management.

Cogan's syndrome is a rare autoimmune disease characterized by the presence of interstitial keratitis and audiovestibular symptoms. The audiovestibular symptoms include fluctuating sensory hearing impairment, tinnitus, vertigo, and reduced vestibular response. Immediate diagnosis and medical intervention provides optimum auditory recovery. Frequent audiologic assessments are necessary to monitor the disease activity and to aid in the therapeutic levels of steroidal medications. Amplification is often required on a temporary or permanent basis. Two case studies are presented to illustrate the audiologist's role in the identification and management of patients with Cogan's syndrome.

Adrenal Cortex Hormones↗

PubMed2026

Whole exome sequencing of paediatric patients with Cogan's syndrome to identify monogenic mimics.

OBJECTIVES: Cogan's syndrome (CS) is a rare variable vessel vasculitis, describing sensorineural hearing loss (SNHL), inflammatory ocular disease and vestibular dysfunction. We hypothesized that within paediatric-onset (p)CS, a proportion would have monogenic disease, either autoinflammatory and/or associated with SNHL. METHODS: Whole exome sequencing (WES) was performed and analysed using an in-house pipeline incorporating virtual gene panels for inflammation and SNHL; copy number variant analysis (ExomeDepth); and phenotype-driven variant prioritization (Exomiser). Genetic variants were interpreted by a multi-disciplinary team according to American College of Medical Genetics and Genomics guidelines. RESULTS: Ten patients with a clinical diagnosis of pCS were enrolled. Three/10 (30%) had a monogenic contribution to the phenotype based on Class 4/5 variants: de novo NLRP3 p.T915R (n = 1) associated with Cryopyrin-associated periodic syndrome; MYO7A p.K542Qfs*5 (n = 1) causing SNHL; and HBB homozygous p.E7V causing sickle cell disease (associated with hearing loss and uveitis). A further two cases had possible monogenic contribution with the following rare variants of uncertain significance (class 3): ADGRV1 compound heterozygous variants (n = 1) associated with Usher syndrome; and a novel ALPK1 p.H735P (n = 1), associated with Retinal dystrophy Optic nerve oedema Splenomegaly Anhidrosis Headache (ROSAH) syndrome. CONCLUSIONS: In children presenting with features suggesting CS, genetic screening should be considered before conferring this rare diagnostic label since at least 30% had an alternative monogenic contribution to the phenotype rather than true pCS, with implications for treatment and prognosis. We thus advocate for genetic testing using next-generation sequencing for patients presenting with pCS.

Humans↗

PubMed1976

Cogan's syndrome: a systemic vasculitis.

Nonsyphilitic interstitial keratitis with vestibuloauditory dysfunction (Cogan's syndrome) is a rare clinical entity. We have reviewed 53 cases (including one of our own) of this disease. In 72 per cent of the affected patients there was an underlying systemic process, often a vasculitis. Ten per cent had fatal or near fatal aortic valvular disease, which has been shown to be amenable to surgical intervention. Other systemic manifestations have included congestive heart failure, gastrointestinal hemorrhage, adenopathy, splenomegaly, hypertension, musculoskeletal involvement and eosinophilia. The clinical course is extremely variable, ranging from months to over 15 years with a minimal five year survival of 28 per cent. Medical therapy with corticosteroids has been beneficial but has only limited effect on symptoms of vestibuloauditory dysfunction. Cogan's syndrome appears to be a manifestation of a systemic disorder which is often apparent only after long-term follow-up.

Adolescent↗

PubMedDate not supplied

Immunological findings in a case of Cogan's syndrome.

The immune system of a case of Cogan's syndrome was investigated. A transient depression of the cell-mediated immunity was found. Thus, during the acute stage of the disease, there was a depression of the cutaneous delayed hypersensitivity reaction to PPD and a decrease in the number of T cells. There were also signs of complement consumption. A possible pathogenesis based on immune complexes due to a preceding viral infection is discussed.

Acute Disease↗

PubMed1978

The HLA antigens in Cogan's syndrome.

HLA typing of ten patients with well documented Cogan's syndrome failed to support previous reports of an increased incidence of HLA-B17. Although HLA-A9 (Aw24), Bw35, and Cw4 appeared increased in frequency among patients, the small number of cases precluded attaching importance to the significance of these increased frequencies.

Adolescent↗

PubMed1992

Corneal autoimmunity in Cogan's syndrome? Report of two cases.

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.

Adult↗

PubMed1978

[Cogan's syndrome--report of a case (author's transl)].

A case of Cogan's syndrom--the combination of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms--is presented. The differential diagnosis of the disease that corresponds little to any treatment, is discussed.

Adult↗

PubMed1977

[Cogans's syndrome in childhood (author's transl)].

A case--report is given of a patient with Cogan's syndrome in childhood. This syndrome consists of non-syphilitic interstitial keratitis and vestibuloauditory symptoms. Visual loss is rare but hearing loss is rapidly progressive. A relation to autoimmune diseases especially to panarteriitis nodosa has been suggested by a number of authors.

Autoimmune Diseases↗

PubMed1975

Iris nevus (Cogan-Reese) syndrome. A cause of unilateral glaucoma.

Fourteen patients (ten women, four men) with iris nevus (Cogan-Reese) syndrome, all having unilateral glaucoma, were studied clinically. Many had corneal edema, and all had changes in the iris consisting of one or more of the following: iris whorls or nodules or both, atrophy of iris stroma, heterochromia (the darker iris usually involved), or ectropion uveae. All had peripheral anterior synechias. Material for histological study was available from ten patients and showed a nonmalignant diffuse nevus of the anterior surface of the iris. An overgrowth of endothelium and Descemet membrane extending onto the iris surface may be a characteristic part of the process. Patients tended to be women in middle age. Conservative treatment avoiding enucleation is advised. Whenever a patient with suspected iris nevus syndrome or essential iris atrophy is subjected to glaucoma surgery, a biopsy specimen of the iris should be obtained for histological study.

Adolescent↗

PubMed1978

Cogan's syndrome with arthritis and aortic insufficiency.

A young man with nonsyphilitic keratitis and vestibulo-auditory dysfunction (Cogan's syndrome) and a recurrent febrile illness has been observed over a 10 year period. The systemic manifestations have included a splenic abscess vasculitis, pericarditis, aortic valvulitis, and mono-articular inflammatory arthritis.

Abscess↗