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Uveomeningoencephalitic Syndrome


Presentation

  • 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]
  • Hearing disturbances (75%) and dizziness may also be present.[orpha.net]
  • 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 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]
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]
  • VKH often becomes bilateral, with patients experiencing blurred vision. “Sometimes there is pain and discomfort,” Dr. Tessler said. Patients also usually develop exudative retinal detachments. In fact, Dr.[healio.com]
Retinal Lesion
  • Relationship between retinal lesions and inward choroidal bulging in Vogt-Koyanagi-Harada disease. Am J Ophthalmol. 2014 ;157: 1056 – 1063 Sakata VM, da Silva FT, Hirata CE, et al.[doi.org]
  • Retinal lesions found in multiple evanescent white dot syndrome can be mistaken with VKHD. MEWDS is usually unilateral, affects young women and is characterized by sudden and painless loss of visual acuity.[doi.org]
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]
  • 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
  • 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]
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]
  • 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]
  • 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]
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]
  • manifestations, including poliosis, vitiligo, and alopecia.[en.wikipedia.org]
  • The disease is characterised by bilateral uveitis associated with a varying constellation of auditory, neurological and cutaneous manifestations. The disease occurs more frequently among people with darker skin pigmentation.[ncbi.nlm.nih.gov]
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]
  • 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]
  • 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]
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]
  • 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]
  • Signs & Symptoms Vogt-Koyanagi-Harada disease is initially characterized by headaches, very deep pain in the eyes, dizziness (vertigo), and nausea.[rarediseases.org]
  • 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
  • manifestation, 054078, 症状, ショウジョウ, symptom, 040039[togodb.biosciencedbc.jp]
  • 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]
  • 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]
  • 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]

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]
HLA-DR4
  • VKH has been linked to human leukocyte antigen DR4 (HLA-DR4) and HLA-Dw53, with strongest associated risk for HLA-DRB1*0405 haplotype.[ncbi.nlm.nih.gov]
  • Genes encoding for human leukocyte antigens (HLAs) have been shown to be associated with certain uveitis entities, including BD (HLA-B51), VKH syndrome (HLA-DR4, DRB1/DQA1), AAU (HLA-B27), and BCR (HLA-A29).[ncbi.nlm.nih.gov]
  • […] 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

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

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.[ncbi.nlm.nih.gov]
  • 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]

Etiology

  • 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]

Epidemiology

  • 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]
  • 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]
Sex distribution
Age distribution

Pathophysiology

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

Prevention

  • […] 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]
  • […] use Centers for Disease Control and Prevention (U.S.)[decs.bvs.br]
  • In the same study, 40.9% of patients with chronic disease had isolated ocular disease. [13] It is speculated that early and aggressive systemic treatment may prevent progression to complete VKH disease. [14] Etiology VKH disease is currently considered[emedicine.com]

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