A 29-year-old man presented with headache and decreased vision in his left eye (LE) for 2 days. Best-corrected visual acuity was 20/20 in both his eyes. [ncbi.nlm.nih.gov]

[…] affecting eyes, meninges, ears and skin Sudden onset of blindness, pleocytosis and temporary hearing loss; no history of eye trauma or surgery (by definition) Blindness is due to severe bilateral panuveitis with serous retinal detachments HLA-DRB1*0405 is present [pathologyoutlines.com]

Patients often do not present to their ophthalmologist until this stage, when they experience acute ocular pain and red eyes associated with bilateral blurring of vision secondary to uveitis. [aao.org]

Hearing disturbances (75%) and dizziness may also be present. [orpha.net]

• Fatigue

  This can lead to a type of fatigue that can only be cur … read more Dr. Rick Board Certified MD Doctoral Degree 12,622 satisfied customers I HAVE DRY EYES, GRANULATED EYE LIDS, BLOOD SHOT EYE. NO I HAVE DRY EYES, GRANULATED EYE LIDS, BLOOD SHOT EYE. [justanswer.com]

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• Nausea

  There may be fever, headache, nausea, meningismus, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard), tinnitus, and/or vertigo. Eye symptoms can include orbital pain, photophobia and tearing. [en.wikipedia.org]

  Signs & Symptoms Vogt-Koyanagi-Harada disease is initially characterized by headaches, very deep pain in the eyes, dizziness (vertigo), and nausea. [rarediseases.org]

  The prodromic stage is characterized by nonspecific symptoms such as fever, headaches, nausea and vertigo, and then by neurologic symptoms such as muscle weakness, hemiparesis, hemiplegia, dysarthria and orbital pain. [orpha.net]

  Initial onset of Vogt-Koyanagi-Harada syndrome is characterized by severe headache, deep pain in the eye, vertigo (feeling that the room is spinning), and nausea, followed in a few weeks by uveitis and vision loss. [healthcentral.com]

• Blurred Vision

  In the ophthalmologic stage, occurring a few days after the prodromic stage, patients complain of blurred vision, ocular pain, and photophobia or central scotoma (bilateral in 80% of cases). [orpha.net]

  A 57-year-old man with no family history of neurologic disease was healthy until age 40 years, when he gradually experienced bilateral eye pain and blurred'' vision. [neurology.org]

  Case Presentation An 18-year old woman with a history of astigmatism presented with the complaint of blurred vision in the right eye for two weeks. [omicsonline.org]

  The main symptom of the acute stage of VKH is blurred vision in both eyes [1]. Ophthalmological findings are panuveitis or posterior uveitis, with multifocal serous retinal detachments [2]. [dermnetnz.org]

• Corneal Opacity

  Vogt-Koyanagi-Harada syndrome - see under Vogt, Alfred Vogt, Alfred, Swiss ophthalmologist, 1879-1943. limbal girdle of Vogt - corneal opacity that occurs in an arc concentric pattern. [medical-dictionary.thefreedictionary.com]

• Tinnitus

  There may be fever, headache, nausea, meningismus, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard), tinnitus, and/or vertigo. Eye symptoms can include orbital pain, photophobia and tearing. [en.wikipedia.org]

  […] multifocal exudative retinal or retinal pigment epithelial detachments, disc hyperemia or edema, or “sunset glow” fundus, which is a yellow-orange appearance of the fundus due to depigmentation of the RPE and choroid, 3) neurologic signs, including tinnitus [aao.org]

  The ocular symptoms were accompanied by tinnitus and marked dyspnea. Pulmonary consultation identified nonspecific pulmonary findings which were out of proportion to her dyspnea. [karger.com]

  During the initial assessment by the Ophthalmologist, she complained about persistent tinnitus and headache, considered as unspecific by the Otorhinolaryngologist and Neurologist. [omicsonline.org]

  Other features include the central nervous system and cutaneous origin, including deafness (sensorineural), headache, tinnitus, dysacousis, and meningism associated with cerebrospinal fluid pleocytosis. [accessanesthesiology.mhmedical.com]

• Dysacusis

  In a retrospective review of 48 patients, Beniz et al. report the prevalence of headache (67%), tinnitus (17%), dysacusis (13%), vitiligo (10%), alopecia (13%), and poliosis (6%). [omicsonline.org]

  Neurologic symptoms tend to occur early and include tinnitus, dysacusis (auditory agnosia), vertigo, headache, and meningismus. [merckmanuals.com]

  […] inflammation Vogt in 1906 • Primary posterior uveitis exudative RDs CSF pleocytosis Einosuke Harada • B/l chronic iridocyclitis skin & hair depigmentation  Koyanagi • This constellation of findings was termed “uveitis with polosis, vitiligo, alopecia and dysacusis [de.slideshare.net]

  Inner ear involvement Changes in the inner ear, such as dysacusis, hearing loss and vertigo, have been observed in 70 % of patients, especially during the prodromal stage. Tinnitus is present in 42 % [ 65 ]. [doi.org]

• Hearing Impairment

  Ocular findings may be accompanied by lymphocytic meningitis, hearing impairment and/or tinnitus in a variable proportion of patients. [doi.org]

• Hearing Problem

  Of the cases of VKH with hearing problems, 75 % returned to normality; however, although very rarely, VKH disease can cause profound hearing loss.11Immunosuppressive therapy The usefulness of IST has been widely demonstrated in patients with autoimmune [elsevier.es]

• Alopecia

  […] depigmented Hair), Hearing [..] 0 0 A syndrome characterized by bilateral granulomatous UVEITIS with IRITIS and secondary GLAUCOMA, premature ALOPECIA, symmetrical VITILIGO, poliosis circumscripta (a strand of depigmented hair), HEARING [..] [dictionary.university]

  Engelsk definition A syndrome characterized by bilateral granulomatous UVEITIS with IRITIS and secondary GLAUCOMA, premature ALOPECIA, symmetrical VITILIGO, poliosis circumscripta (a strand of depigmented hair), HEARING DISORDERS, and meningeal signs [mesh.kib.ki.se]

  A syndrome characterized by bilateral granulomatous UVEITIS with IRITIS and secondary GLAUCOMA, premature ALOPECIA, symmetrical VITILIGO, poliosis circumscripta (a strand of depigmented hair), HEARING DISORDERS, and meningeal signs (neck stiffness and [ncbi.nlm.nih.gov]

  VKH is a multisystem disorder and includes idiopathic uveitis, dysacousia, leukodermia, alopecia, canities (whitened hair), poliosis (whitened eyebrows and eyelashes), acute encephalitic signs, and fluctuating meningeal symptoms both central and peripheral [jamanetwork.com]

  It is manifested with hearing disorders, panuveitis, alopecia, poliosis, vitiligo, neck stiffness, headaches, and focal neurologic signs ICD-9-CM Volume 2 Index entries containing back-references to 364.24 : [icd9data.com]

• Cutaneous Manifestation

  Given that the cutaneous manifestations of VKH disease are so characteristic, these could not have been missed even before the actual clinical entity of VKH was recognized in the early 20th century. [ncbi.nlm.nih.gov]

  manifestations, including poliosis, vitiligo, and alopecia. [en.wikipedia.org]

  Vogt-Koyanagi-Harada syndrome is a multisystem disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. [radiopaedia.org]

• Piebaldism

  The cutaneous symptoms may lead us to consider diagnostic possibilities such as Alezzandrini's syndrome, alopecia areata, vitiligo and piebaldism 10. [scielo.br]

  Besides that, the cutaneous manifestations should exclude diagnosis such as Alezzandrini’s syndrome, alopecia areata, vitiligo and piebaldism. Other immunomediated sensorineural hearing loss should also be excluded such as Cogan syndrome. [doi.org]

• Meningism

  Examination of the cerebrospinal fluid reveals a pattern consistent with MENINGITIS, ASEPTIC. [mesh.kib.ki.se]

  Examination of the cerebrospinal fluid reveals a pattern consistent with meningitis, aseptic. [icd9data.com]

  Definition / general Also called Vogt-Koyanagi-Harada syndrome Rare multisystemic autoimmune disease affecting eyes, meninges, ears and skin Sudden onset of blindness, pleocytosis and temporary hearing loss; no history of eye trauma or surgery (by definition [pathologyoutlines.com]

  VKH is a multisystem disorder and includes idiopathic uveitis, dysacousia, leukodermia, alopecia, canities (whitened hair), poliosis (whitened eyebrows and eyelashes), acute encephalitic signs, and fluctuating meningeal symptoms both central and peripheral [jamanetwork.com]

• Headache

  A 29-year-old man presented with headache and decreased vision in his left eye (LE) for 2 days. Best-corrected visual acuity was 20/20 in both his eyes. [ncbi.nlm.nih.gov]

  […] syndrome characterized by bilateral granulomatous UVEITIS with IRITIS and secondary GLAUCOMA, premature ALOPECIA, symmetrical VITILIGO, poliosis circumscripta (a strand of depigmented hair), HEARING DISORDERS, and meningeal signs (neck stiffness and headache [mesh.kib.ki.se]

  It is manifested with hearing disorders, panuveitis, alopecia, poliosis, vitiligo, neck stiffness, headaches, and focal neurologic signs ICD-9-CM Volume 2 Index entries containing back-references to 364.24 : [icd9data.com]

  The prodromic stage is characterized by nonspecific symptoms such as fever, headaches, nausea and vertigo, and then by neurologic symptoms such as muscle weakness, hemiparesis, hemiplegia, dysarthria and orbital pain. [orpha.net]

• Vertigo

  There may be fever, headache, nausea, meningismus, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard), tinnitus, and/or vertigo. Eye symptoms can include orbital pain, photophobia and tearing. [en.wikipedia.org]

  Signs & Symptoms Vogt-Koyanagi-Harada disease is initially characterized by headaches, very deep pain in the eyes, dizziness (vertigo), and nausea. [rarediseases.org]

  The prodromic stage is characterized by nonspecific symptoms such as fever, headaches, nausea and vertigo, and then by neurologic symptoms such as muscle weakness, hemiparesis, hemiplegia, dysarthria and orbital pain. [orpha.net]

  Initial onset of Vogt-Koyanagi-Harada syndrome is characterized by severe headache, deep pain in the eye, vertigo (feeling that the room is spinning), and nausea, followed in a few weeks by uveitis and vision loss. [healthcentral.com]

• Neurologic Manifestation

  In this update, the author cites work that shows that earlier initiation of either glucocorticoids or immunomodulatory measures significantly reduces late visual morbidity in patients with Vogt-Koyanagi-Harada syndrome and that the neurologic manifestations [medlink.com]

  Patients must have either the neurologic manifestations or dermatological signs. c. Probable VKH disease: similar eye disease as patients with complete VKH disease but without neurologic and dermatological signs. [rarediseases.org]

  manifestation, 054078, 症状, ｼｮｳｼﾞｮｳ, symptom, 040039 [togodb.biosciencedbc.jp]

  Complications of the disorder include the following: Cataract Subretinal fibrosis Choroidal neovascularization Neovascularization of the disc Pigmentary changes of the fundus Optic atrophy Neurologic manifestations - Many of the neurologic manifestations [emedicine.com]

• Dizziness

  Hearing disturbances (75%) and dizziness may also be present. [orpha.net]

  Hearing disturbances and dizziness may be present. After weeks or months, most patients notice cutaneous signs (e.g. hair loss, poliosis, vitiligo). [radiopaedia.org]

  Signs & Symptoms Vogt-Koyanagi-Harada disease is initially characterized by headaches, very deep pain in the eyes, dizziness (vertigo), and nausea. [rarediseases.org]

  The diagnosis of clinical deafness, tinnitus and dizziness should always consider Vogt-Koyanagi-Harada syndrome. [arquivosdeorl.org.br]

(See Presentation and Workup.) [6, 7, 8, 9, 10] Bilateral, multifocal serous detachments in a patient with Vogt-Koyanagi-Harada disease. Disc hyperemia is evident in the right eye. [emedicine.com]

Exudative reti- TABLE 1 Diagnostic workup for Vogt-Koyanagi-Harada disease nal detachments in uveal effusion syndrome tend to be chronic and do not respond to corticosteroid treatment. [slideshare.net]

• HLA-DR4

  The association of HLA-DR4/ HLA-DRB1*04 with VKH was reported in various ethnic populations. Our meta-analysis confirms these previous reports on the association of HLA-DR4/ HLA-DRB1*04 with VKH. [nature.com]

  […] found an association with HLA-DR4 and HLA-DR53, while HLA-DR1 and HLA-DR4 were reported in Hispanic patients living in southern California. [40] An association with HLA-DR4, HLA-DRw53, and HLA-DQw3 has been found in subjects of Native American ancestry [emedicine.com]

  VKH has been linked to human leukocyte antigen DR4 (HLA-DR4) and HLA-Dw53, with strongest associated risk for HLA-DRB1*0405 haplotype. [doi.org]

Immediate high-dose systemic corticosteroid treatment was given. [ncbi.nlm.nih.gov]

Standard Therapies Treatment Standard treatment of Vogt-Koyanagi-Harada disease is the use of high-dose systemic steroid drugs initially and often followed by immunosuppressive therapy. [rarediseases.org]

Management and treatment Management should involve a multidisciplinary team with dermatologists, ophthalmologists, and neurologists. High doses of corticosteroids usually provide efficient treatment, but immunosuppressors may also be used. [orpha.net]

With treatment, the visual outcome of VKH is generally good. [aao.org]

Related to treatment: a.. Late instauration of treatment from the acute disease onset : early treatment with high-dose systemic corticosteroids resulted in less persistent inflammation [ 176 ]; b.. [doi.org]

Prognosis With early and aggressive treatment, the prognosis is usually favorable, but acute hearing and vision alterations may occur. [orpha.net]

Visual prognosis is generally good with prompt diagnosis and aggressive immunomodulatory treatment. [doi.org]

Visual prognosis is generally good with prompt diagnosis and aggressive immunomodulatory treatment. Inner ear symptoms usually respond to corticosteroid therapy within weeks to months; hearing usually recovers completely. [en.wikipedia.org]

Prognosis The important action for a positive prognosis is an immediate start of therapy by taking high doses of anti-inflammatory medication in order to control inflammation (40). [arquivosdeorl.org.br]

Year introduced: 1991(1965) PubMed search builder options Subheadings: analysis anatomy and histology blood cerebrospinal fluid chemically induced classification complications diagnosis diagnostic imaging drug therapy enzymology epidemiology ethnology etiology [ncbi.nlm.nih.gov]

Etiology Pathogenesis has been linked to immunological disorders targeting the melanocytes, and which involve T-cell-mediated cytotoxicity and apoptosis. However, the etiology is still not completely understood. [orpha.net]

Diagnosis - Etiology en_US dc.subject.mesh Male en_US dc.subject.mesh Uveitis - Diagnosis - Etiology en_US dc.subject.mesh Uveomeningoencephalitic Syndrome - Complications - Drug Therapy en_US dc.title High-grade lymphoma after azathioprine treatment [hub.hku.hk]

Subject Adult; Evoked Potentials, Visual; Female; Humans; Pilocarpine/administration & dosage; Pupil/drug effects; Tonic Pupil/etiology; Uveomeningoencephalitic Syndrome/complications/diagnosis; Visual Acuity Abstract We report a 38-year-old woman with [collections.lib.utah.edu]

The etiology is unknown, but it may be an inflammatory autoimmune condition. Called also Harada syndrome. [medical-dictionary.thefreedictionary.com]

1995 Jan;39(4):265-292) Year introduced: 1991(1965) PubMed search builder options Subheadings: analysis anatomy and histology blood cerebrospinal fluid chemically induced classification complications diagnosis diagnostic imaging drug therapy enzymology epidemiology [ncbi.nlm.nih.gov]

Summary Epidemiology Annual incidence is estimated at 1/400,000. The condition predominantly affects young women and individuals with darker pigmentation (those of Asian, Hispanic, or Native American origin). [orpha.net]

Sciences Toxoplasmosis Medicine & Life Sciences Uveomeningoencephalitic Syndrome Medicine & Life Sciences Uveitis Medicine & Life Sciences Ocular Hypotension Medicine & Life Sciences Iris Medicine & Life Sciences Projects 2016 2018 3 Finished Zika virus epidemiology [pure.urosario.edu.co]

(See Etiology and Epidemiology.) [1, 2, 3, 4, 5] Independently, Vogt, Koyanagi, and Harada described several patients during a 20-year period with bilateral uveitis, exudative retinal detachments, neurologic abnormalities, and disorders of the integument [emedicine.com]

[…] syndrome was first reported in the 10th century by Ali Ibn Isa, an ophthalmologist in medieval Baghdad, who described the characteristic physical appearance of ocular inflammation with a distinct whitening of the hair, eyebrows, and eyelashes. 1 The pathophysiology [ajnr.org]

The underlying pathophysiology involves an immune-mediated attack of pigmented cells in the affected organ systems. Consequently, VKH syndrome is more common in individuals with darker skin pigmentation. [omicsonline.org]

[…] fibrosis; less frequently, posterior synechiae, neovascularization of the optic disc, arteriovenous anastomoses and choroidal neovascularization occur.4,6,7 All these complications severely compromise the visual prognosis.Etiology and pathogenesis The pathophysiology [elsevier.es]

It may also contribute to the understanding of the pathophysiology of chorioretinal inflammatory disordes [ 99 ]. [doi.org]

[…] histology blood cerebrospinal fluid chemically induced classification complications diagnosis diagnostic imaging drug therapy enzymology epidemiology ethnology etiology genetics history immunology metabolism microbiology pathology physiology physiopathology prevention [ncbi.nlm.nih.gov]

Treatment is important and can prevent serious complications. Make sure you report any new symptom to your doctor, and go to your follow-up visits as your doctor says. [webmd.com]

Others • Patients with inflammatory cell infiltration in the anterior chamber require topical corticosteroids and cycloplegics to reduce ciliary spasm and prevent posterior synechiae formation 55. [de.slideshare.net]

Early recognition and treatment can prevent progression to chronic convalescent and chronic recurrent stages of the disease [7]. [dermnetnz.org]