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Uveomeningoencephalitic Syndrome
Syndrome Uveomeningoencephalitic

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WIKIDATA, CC BY 2.0
WIKIDATA, Public Domain

Presentation

To present revised criteria for the diagnosis of Vogt-Koyanagi-Harada disease, a chronic, bilateral, granulomatous ocular and multisystem inflammatory condition of unknown cause. [ncbi.nlm.nih.gov]

Patients are often typically middle-aged (range of 20-50 years old) at presentation 7. [radiopaedia.org]

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]

An 80-year-old man presented with poliosis that developed suddenly after having surgery for macular degeneration of his right eye about 10 years ago. [kyushu-u.pure.elsevier.com]

Patients can present skin and hair sensitiveness. For some period though, patients might not present such symptoms or atypical signs as optical neuritis and paralysis of the peripheral nerve (1,2,18). [arquivosdeorl.org.br]

Entire Body System

  • Malaise

    In this same time period, Einosuke Harada, in an article published in Nippon Ganka Gakkai Zasshi in 1926 that was based on several case studies, comprehensively described a syndrome that included (1) a prodromal phase of malaise and meningeal irritation [ncbi.nlm.nih.gov]

    […] sign other ocular signs: nummular chorioretinal depigmented scars, or retinal pigment epithelium clumping and/or migration, or recurrent or chronic anterior uveitis neurological/auditory findings (may have resolved by time of examination) meningismus (malaise [radiopaedia.org]

    The eye symptoms may be accompanied by a varying constellation of systemic symptoms, such as auditory (tinnitus, vertigo, and hypoacusis), neurological (meningismus, with malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back [en.wikipedia.org]

    Meningismus (malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a combination of these factors; headache alone is not sufficient to meet definition of meningismus, however), or b. Tinnitus, or c. [eyewiki.aao.org]

  • Fatigue

    This can lead to a type of fatigue that can only be cur … read more Dr. Rick Board Certified MD Doctoral Degree 13,344 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]

    A few of the illnesses helped by osteopathic manipulation are spinal and joint conditions, arthritis, allergies, cardiac diseases, breathing dysfunctions, chronic fatigue syndrome, hiatal hernias, high blood pressure, headaches, digestive disorders, bladder [alternative-health-group.org]

  • Constitutional Symptom

    She had a 12-day history of headache, tinnitus, earache, non-productive cough, rhinorrhea, epistaxis and dyspnea together with constitutional symptoms of night sweats and weight loss. [karger.com]

Gastrointestinal

  • 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]

  • Abdominal Pain

    pain, stiffness of the neck and back, or a combination of these factors, but headache alone is insufficient), or tinnitus, or cerebrospinal fluid pleocytosis integumentary finding (not preceding onset of central nervous system or ocular disease) alopecia [radiopaedia.org]

    The eye symptoms may be accompanied by a varying constellation of systemic symptoms, such as auditory (tinnitus, vertigo, and hypoacusis), neurological (meningismus, with malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back [en.wikipedia.org]

    Meningismus (malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a combination of these factors; headache alone is not sufficient to meet definition of meningismus, however), or b. Tinnitus, or c. [eyewiki.aao.org]

    Neurological/auditory findings (may have resolved by time of examination) a) Meningismus (malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a combination of these factors; headache alone is not sufficient to meet the [de.slideshare.net]

Eyes

  • 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. [n.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]

  • Lacrimation

    (Lacrimal Apparatus Diseases) Arcus Senilis Arcus, Corneal (Arcus Senilis) Asthenopia Astigmatism Astrocytoma, Malignant, Optic Nerve (Optic Nerve Glioma) Atrophies, Gyrate (Gyrate Atrophy) Atrophies, Hereditary Optic (Optic Atrophies, Hereditary) Atrophy [provisu.ch]

    Photophobia and lacrimation may develop, and also feelings of hypersensitivity in the skin and hair. [scielo.br]

Ears

  • Tinnitus

    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]

    […] 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]

    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]

    Case 3 A 16-year-old Asian Indian woman presented with decreased vision in each eye, severe headache, and tinnitus of 2 weeks’ duration. Vision was 2/60 in the right eye and counting fingers in the left. [bjo.bmj.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. [msdmanuals.com]

    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

    She had profound hearing impairment bilaterally. She also had dysdiadochokinesia and dysmetria on the right. [nn.neurology.org]

    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]

Skin

  • Alopecia

    Vogt-Koyanagi syndrome - bilateral uveitis with iritis and glaucoma, premature graying of the hair, alopecia, vitiligo, and dysacusia. [medical-dictionary.thefreedictionary.com]

    […] 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]

    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]

    Alopecia-Poliosis-Uveitis-Vitiligo-Deafness-Cutaneous-Uveo-Oto Syndrome; Harada Syndrome; Harada Disease; Vogt-Koyanagi Syndrome; Yugé Syndrome; Oculocutaneous Syndrome; Uveocutaneous Syndrome; Uveitis-Vitiligo-Alopecia-Poliosis Syndrome; Uveomeningeal [accessanesthesiology.mhmedical.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]

    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]

    Requires three of the following features: bilateral iridocyclitis, posterior uveitis (including serous retinal detachment or sunset glow fundus), central nervous system manifestations, and cutaneous manifestations. [accessanesthesiology.mhmedical.com]

  • 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]

Neurologic

  • Meningism

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

    Historical note and terminology The subject of this review is a clinical syndrome in which pigmentary alterations in various tissues are associated with ocular and meningeal inflammation. [medlink.com]

    [Article in Spanish] PMID: 13841515 Case Reports [Uveo-meningitis, uveo-encephalitis: Vogt-Koyanagi-Harada syndrome. Intracranial pseudohypertension. Report of 3 cases] [Article in Spanish] A VAZQUEZ LEYVA. Arch Hosp Univ. 1959 Jul-Aug. [pubmed.ncbi.nlm.nih.gov]

    The case 1 was a white Brazilian, forty-five year-old man, with a acute headache, mental confusion and signs of meningeal irritation. [scielo.br]

    We diagnosed recurrent Vogt-Koyanagi-Harada disease (VKH) ; however, systemic corticosteroid therapy was not carried out, because there were no signs of acute inflammation such as meningism, dysacousis, or retinal detachment. [kyushu-u.pure.elsevier.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]

    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 patient was given prednisolone acetate eye drops and oral prednisone, and his vision and headaches gradually improved during the next several weeks. By 6 months, the headaches did not recur and the visual acuity did not decline further. [ajnr.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]

    CASE REPORTS Case 1 A 37-year-old Asian Indian woman presented with a 4 day history of bilateral eye pain, redness, decreased vision, and headache. Vision was right eye 6/36 and 6/6 left eye. [bjo.bmj.com]

  • 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]

    manifestation, 054078, 症状, ショウジョウ, symptom, 040039 [togodb.biosciencedbc.jp]

    Neurologic manifestations of VKH typically include aseptic meningitis and headache. Focal neurologic signs such as cranial nerve palsies, hemiparesis, and optic neuritis are relatively uncommon. [nn.neurology.org]

    Incomplete VKH disease: similar eye disease as patients with complete VKH disease but do not have both neurologic and dermatological signs. Patients must have either the neurologic manifestations or dermatological signs. c. [rarediseases.org]

  • 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]

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

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

Workup

(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 Type

  • 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]

    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]

    […] 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]

Treatment

This anti-TNF agent seemed to be an effective and well-tolerated treatment for these patients. [iovs.arvojournals.org]

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]

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

Prognosis

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]

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]

Etiology

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]

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]

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

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]

Epidemiology

This review focuses on the diagnostic criteria of VKH disease, including some information on history, epidemiology, appropriate clinical and classification criteria, etiopathogenesis, treatment and outcomes. [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]

Pathophysiology

Vogt-Koyanagi-Harada disease: Novel insights into pathophysiology, diagnosis and treatment. [ciencialatina.org]

[…] 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]

Prevention

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]

[…] 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]

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

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