Cogan’s syndrome (CS) is a rare inflammatory disorder characterized by interstitial keratitis and vestibuloauditory abnormalities often associated with various systemic manifestations. Involvement of cardiovascular system resembling systemic vasculitis may lead to severe complications and death. The present report describes a case of a female patient with atypical Cogan's syndrome presented with systemic manifestations and severe coronary and femoral artery stenosis. Despite the clinical improvement after glucocorticoids and cyclophosphamide, the patient required double aortocoronal bypass grafting one year letter. During three years follow-up, she was in stable condition, without stenocardial symptoms and claudication and her inflammatory parameters remain normal. This case highlights the rare involvement of coronary arteries without associated large-vessel vasculitis of the aortic arch in CS.
Adult ; Cogan Syndrome ; complications ; Coronary Disease ; etiology ; Female ; Humans

Cogan's syndrome is a rare systemic inflammatory disease and can be diagnosed on the basis of typical inner ear and ocular involvement with the presence of large vessel vasculitis. We report a case of Cogan's syndrome with stable angina resulting from coronary ostial stenosis caused by aortitis.
Angina Pectoris ; Angina, Stable ; Aortitis ; Cogan Syndrome ; Constriction, Pathologic ; Ear, Inner ; Glycosaminoglycans ; Vasculitis

Congenital ocular motor apraxia is a rare syndrome characterized by rotational head thrusts that attempt to compensate for the lack of voluntary eye movements. We describe the clinical, oculographic and magnetic resonance imaging features of two children with congenital ocular motor apraxia.
Apraxias ; Child ; Cogan Syndrome ; Eye Movements ; Head ; Humans ; Magnetic Resonance Imaging

Cogan's syndrome is a rare chronic inflammatory disease Characterized by non-syphilitic keratitis and vestibuloauditory dysfunction. Although the precise pathogenesis of Cogan's syndrome is unknown, it is thought to develop from vasculitis involving multiple organ system. It can be accompanied with various systemic diseases including arthritis, lymphadenopathy, splenomegaly, and aortitis with insufficiency. We report a case of typical Cogan's syndrome with multiple cerebral infarctions.
Aortitis ; Arthritis ; Cerebral Infarction ; Cogan Syndrome ; Keratitis ; Lymphatic Diseases ; Splenomegaly ; Vasculitis

BACKGROUND: Congenital ocular motor apraxia (COMA) is characterized by impaired voluntary saccades and abnormal head thrusts. However, mechanism of this disorder remains to be elucidated. METHODS: This study analyzed the eye movements and imaging findings in 16 patients with COMA, who had been recruited from 2003 to 2009 at the Neuro-Ophthalmology Clinic of Seoul National University Bundang Hospital. RESULTS: All the children showed impaired saccades and smooth pursuit in the horizontal direction. One of them also exhibited impaired vertical saccades and smooth pursuit. Eight children showed excessive blinks in association with an attempt to generate saccades. The typical head thrust usually developed around the age of eight months and had resolved by the age of 6-7 years. History of spasmus nutans was confirmed in seven children. Fourteen children showed cerebellar vermian hypoplasia, mostly in the inferior portion, and five of them also had dysgenesis of the corpus callosum. The severity of cerebellar vermian hypoplasia was correlated with developmental delay, as determined by the age of independent walking. CONCLUSION: Cerebellar vermian hypoplasia is a frequent finding in COMA. Dysfunction of the oculomotor vermis may responsible for the impaired saccades and smooth pursuit in COMA. The occasional association of COMA with spasmus nutans indicates a common pathophysiology of these benign developmental disorders.
Apraxias ; Cerebellum ; Child ; Cogan Syndrome ; Coma ; Corpus Callosum ; Eye Movements ; Head ; Humans ; Infant ; Infant, Newborn ; Pursuit, Smooth ; Saccades ; Spasms, Infantile

Cogan's syndrome is a rare chronic inflammatory disease of unknown origin, characterized by nonsyphilitic interstitial keratitis, vestibuloauditory dysfunction and vasculitis. Cogan's syndrome is uncommon and few cases have been published. A case of Cogan's syndrome in a patient with anti-Ro and anti-La positivity is described. A 24-year-old woman visited to department of rheumatology with dry mouth, dry eye, and vertigo on August 2004, and interstitial keratitis had developed on October 2004. She was admitted to the otolaryngology department with rapidly progressive hearing loss on December 2004. The patient's audiogram revealed severe sensorineural deafness. The patient was treated with systemic corticosteroid and methotrexate. This case is the first to report a case of Cogan's syndrome associated with anti-Ro and anti-La positivity.
Antibodies* ; Cogan Syndrome* ; Deafness ; Female ; Hearing Loss ; Humans ; Keratitis ; Methotrexate ; Mouth ; Otolaryngology ; Rheumatology ; Vasculitis ; Vertigo ; Young Adult

Cogan syndrome is a rare inflammatory disease characterized by intraocular inflammation and vestibulo-auditory dysfunction. The exact etiology of Cogan syndrome is still unknown, but is currently thought to be an autoimmune disease. Cogan syndrome can be accompanied with various conditions including fever, arthritis, skin rash, aortitis, central or peripheral nerve system involvement, lymphadenopathy, splenomegaly and diarrhea. We report a case of Cogan syndrome accompanied with meningitis.
Aortitis ; Arthritis ; Autoimmune Diseases ; Cogan Syndrome ; Diarrhea ; Exanthema ; Fever ; Inflammation ; Lymphatic Diseases ; Meningitis ; Peripheral Nerves ; Splenomegaly ; Vasculitis

PURPOSE: To report a case of atypical Cogan syndrome with late-onset corneal involvement. METHODS: Bilateral conjunctival injection with severe headache developed in a 38-years old woman who had suffered from tinnitus, vertigo and hearing difficulty. Inflammation in anterior chamber and vitreous cavity, macular edema, disc edema and retinal hemorrhages were found on ocular examination. RESULTS: After initiation of systemic steroid therapy, ocular inflammatory reaction and visual acuity improved, but hearing difficulty did not respond. After 7 years of lost follow-up, corneal stromal opacity occurred and she was diagnosed as atypical Cogan syndrome. Elevated ESR, multiple stenosis in renal and lower extremity arteries in angiography were found. CONCLUSIONS: Atypical Cogan syndrome with late-onset corneal involvement is rare and difficult to diagnose, which may delay in initiation of adequate treatment.
Adult ; Angiography ; Anterior Chamber ; Arteries ; Cogan Syndrome* ; Constriction, Pathologic ; Edema ; Female ; Follow-Up Studies ; Headache ; Hearing ; Humans ; Inflammation ; Lower Extremity ; Macular Edema ; Retinal Hemorrhage ; Tinnitus ; Vertigo ; Visual Acuity

The syndrome of nonsyphilitic interstitial keratitis associated with vestibulcauditory symptoms (tinnitus, vertigo, nystagmus and progressive deafness) was described by Dr. Cogan in 1945. Since then, numerous cases of this clinical entity have been reported elsewhere in the world. Recently the author experienced one case of Cogan's syndrome in our ophthalmologic OPD for the first time in Korea. The 45 years old male patient, looking very well on gross appearance, was admitted to ENT ward with sudden onset of vertigo, tinnitus and progressive deafness under the diagnosis of Meniere's disease on May 14,1976. Two month later, on July 6, 1976, the visual acuity in his left eye had suddenly deteriorated from 1.2 to 0.6. Under the slit lamp microscopic examination, mild infiltration in the corneal stroma and almost total pcsterior synechia were detected, and a few inflammatory cells were found in anterior chamber but not observed agueous flares. Several fine keratic precipitates were adhered on the lower one half of the posterior corneal surface as a linear apperance. The patient was treated by local instillation of 1% Atropine, 10% Phenylephrine and 3%. Dexamethasone, and also 1% Depomedrol was injected subconjunctivally. Oral administration of Dexamethasone, Vitamine B complex, INH and Chloramphenicol were combined. Thereafter those ocular symptoms had gradually improved, and recently his visual acuity has recovered to normal level (OS 1.2). The vestibuloauditory symptoms just as tinnitus and vertigo were also disappeared, where as moderate deafness has been still remained.
Administration, Oral ; Anterior Chamber ; Atropine ; Chloramphenicol ; Cogan Syndrome* ; Corneal Stroma ; Deafness ; Dexamethasone ; Diagnosis ; Humans ; Keratitis* ; Korea ; Male ; Meniere Disease ; Middle Aged ; Phenylephrine ; Tinnitus ; Vertigo ; Visual Acuity ; Vitamins

Animals ; Apraxias ; congenital ; Cogan Syndrome ; diagnosis ; etiology ; Coma ; Diagnosis, Differential ; Humans ; Malaria, Cerebral ; complications ; diagnosis ; parasitology ; Male ; Middle Aged ; Plasmodium falciparum ; isolation & purification ; Tomography, X-Ray Computed