Achados audiológicos na Síndrome de Vogt-Koyanagi-Harada / Audiological findings in the Vogt-Koyanagi-Harada Syndrome

RESUMO A síndrome de Vogt-Koyanagi-Harada (SVKH) é rara, multissistêmica e autoimune. Atinge principalmente os olhos, provocando uma panuveíte crônica bilateral, porém traz afecções em outras áreas e tecidos que são ricos em melanócitos, como olhos, orelha interna, meninges e a pele. Sua origem ainda não é totalmente conhecida. Geralmente, a SVKH atinge indivíduos de origem hispânica, do Oriente Médio, indianos, nativos americanos e asiáticos. Descrição dos aspectos audiológicos acometidos pela síndrome e as possíveis intervenções fonoaudiológicas para um caso específico. Paciente de 53 anos, sexo feminino, compareceu à Clínica de Fonoaudiologia, Faculdade de Odontologia de Bauru (FOB) com queixas audiológicas e diagnóstico médico da SVKH. A paciente apresentou perda auditiva sensório-neural bilateralmente, emissões otoacústicas evocadas ausentes e queixas vestibulares de vertigem postural e desequilíbrio ao andar, bem como queixa de zumbido agudo contínuo. O caso apresentado mostrou perda auditiva sensório-neural, vertigem, zumbido e acometimento ocular bilateral. Apesar do tratamento com corticoesteroide, a perda auditiva se manteve. Desta forma, precedente à indicação do AASI, o fonoaudiólogo deve atentar-se para o acompanhamento audiológico do caso, realização ou não de tratamento medicamentoso e ocorrência de sintomas sugestivos da síndrome, favorecendo o encaminhamento para o médico e participando ativamente do processo terapêutico envolvendo a audição.

ABSTRACT The Vogt-Koyanagi-Harada syndrome (VKHS) is a rare, multisystemic and autoimmune disease. The syndrome mainly affects the eyes, followed by bilateral chronic panuveitis, however, the syndrome may also affect the melanocytes tissues, for example, the eyes, inner ear, meninges and skin. The syndrome origin mechanism is not yet completely known. Commonly, the specific ethnic groups that are affected by the VKHS are as follows: Hispanics, Asians, Indians, Native Americans and ethnic groups from the Middle East. The audiological characteristics of the syndrome and the possible audiologist interventions for a specific case will be reported. The patient was attended at the Clinic of Speech Therapy, Faculdade de Odontologia de Bauru (FOB). She is 53 years old and presented audiological complaints. She was diagnosed with VKHS by a specialist doctor. Throughout the audiologist assessment, she presented bilateral sensorineural hearing loss, absent otoacoustic evoked emissions, complaints about postural vertigo and acute tinnitus. The specific case reported presented sudden sensorineural hearing loss, vertigo, tinnitus and bilateral ocular disease. Even though drug treatment was performed, the hearing loss remained. Therefore, before the hearing aid (HA) fitting, the audiologist should perform the hearing management, investigate if the patient takes the drug treatment and the occurrence of suggestive symptoms of the syndrome. These are some points that help in the reference to the specialist doctor and the audiologist strongly participates in what concerns the hearing.

Descolamento seroso de coroide como manifestação tardia da síndrome de Vogt-Koyanagi-Harada / Serous choroidal detachment in chronic stage of Vogt-Koyanagi-Harada syndrome

Resumo Relatamos um caso atípico de uma paciente de 40 anos com apresentação completa da Síndrome de Vogt-Koyanagi-Harada (SVKH) que após 17 anos do diagnóstico inicial evoluiu com descolamento seroso de coroide. A paciente procurou atendimento com queixa de dor em olho esquerdo (OE). O exame oftalmológico revelou acuidade visual (AV) igual a de movimento de mãos, à biomicroscopia foi observada reação inflamatória granulomatosa na câmara anterior, a tonometria foi igual a 0 mmhg, e a fundoscopia indevassável pela pouca midríase e turvação de meios em OE. O descolamento seroso de coroide foi avaliado através de ultrassonografia ocular. A abordagem terapeutica intituida para paciente consistiu em prednisona 1mg/kg/dia via oral, dexametasona 1mg/mL e atropina 1% colírios. A evolução do quadro foi satisfatória, com melhora da AV para 20/40, ausência de reação inflamatória em câmara anterior, normalização da pressão intraocular e resolução do descolamento seroso de coroide em OE. Concluimos que a fase crônica da SVKH, apesar da manifestação classicamente descrita ser uveíte anterior, pode ter outras apresentações e o descolamento seroso da coroide é uma rara complicação.

Abstract We report an atypical case of a 40-year-old woman with complete presentation of Vogt-Koyanagi-Harada Syndrome (VKH) who presented with unilateral serous choroidal detachment 17 years after the diagnosis. The patient complained of pain in the left eye, the ophthalmologic examination revealed visual acuity (VA) equal to hand motion; biomicroscopy revealed a granulomatous inflammatory reaction in the anterior chamber, tonometry was equal to 0 mmhg, and the fundoscopy was impracticable. Serous choroidal detachment was assessed by ocular ultrasonography. The therapeutic approach proposed for the patient consisted of prednisone 1mg / kg / day orally, dexamethasone 1mg / mL and atropine 1% eye drops. The evolution of the condition was satisfactory, with VA improvement to 20/40, absence of inflammatory reaction in anterior chamber, normalization of intraocular pressure and resolution of serous choroidal detachment in OS. We conclude that the chronic phase of VKH, although classically described as anterior uveitis, may have other presentations and the serous choroid detachment is a rare complication.

Síndrome de Vogt-Koyanagi-Harada: Avaliação da fase da doença na qual os pacientes recebem o primeiro atendimento em serviço especializado / Vogt-Koyanagi-Harada syndrome: evaluation of the disease phase in which patients receive the first specialized attendance

Resumo Objetivo: Avaliar em qual fase da síndrome de Vogt-Koyanagi-Harada (SVKH) os pacientes recebem o primeiro atendimento em serviço especializado. Métodos: Foram analisados prontuários de 14 pacientes atendidos no Setor de Uveítes do Hospital Universitário Clementino Fraga Filho da UFRJ no período de janeiro de 2014 a março de 2017. Nesta análise, foram observados o sexo, a idade, a fase da doença e a acuidade visual destes pacientes com ao menos doença provável da SVKH. Resultados: Observamos que 35,4% dos pacientes apresentavam a doença ainda na fase uveítica e que 78,5% destes pacientes apresentava acuidade visual igual ou pior que 0,05. Destes pacientes, 78,5% eram do sexo feminino e 21,5% do sexo masculino e a mediana de idades foi de 34 anos. Conclusão: Os pacientes analisados obtiveram dificuldade em ter acesso precoce a um setor especializado, afetando assim, diretamente o tratamento e prognóstico visual.

Abstract Objective: To evaluate in which phase of Vogt-Koyanagi-Harada (VKH) syndrome the patients receive the first attendance in specialized service. Methods: A retrospective study was conduted to evaluate medical records of 14 patients with VKH in the Clementino Fraga Filho University Hospital of the Federal University of Rio de Janeiro from January 2014 to March 2017. In this analysis, gender, age, stage of disease and visual acuity of these patients with at least probable VKH were recorded. Results: Of these patients, 78.5% were female and 21.5% male and the median age was 34 years. We observed that 35.4% of the patients had the disease still in the uveitic phase and that 78.5% of these patients had visual acuity equal to or worse than 0.05. Conclusion: There is a delay in the admission of these patients to a specialized sector, thus affecting directly the treatment and visual prognosis.

Síndrome de Vogt-Koyanagi-Harada en niños / Vogt-Koyanagi-Harada syndrome in children

Vogt-Koyanagi-Harada (VKH) syndrome is a systemic inflammatory disease that causes chronic and bilateral granulomatous panuveitis, usually described in adults. Objectives: To describe manifestations and complications of VKH in pediatric patients. Methods: Retrospectivedescriptive study upon patients <14 years-old with VKH, attended from January 1985 to July 2010 in three different centers. Results: A total of 17 patients (34 eyes) were studied; 9 (53%) female. The mean age was 10.8 years-old. Among extraocular manifestations; neurological (71%), dermatological (29%) and auditive (24%) signs were observed. Ocular findings included optic-disc involvement (94%), anterior uveitis (79%), choroiditis (77%), serous retinal detachment (71%) and vitritis (71%). Initial visual acuity (VA) was ≤0.05 in 47% of cases and ≥0.6 in 12% of patients. 71% presented complications: glaucoma (20 eyes), sinechiae (10 eyes), maculopathy (6 eyes) cataract (5 eyes) and ptisis bulbi (1 eyes). 35% received only corticosteroids and 65% inmunosupressive drugs. After treatment, 6% had VA ≤0.05 and 59% ≥0.6. Ten patients (59%) recurred: 30% compromising posterior pole, and 50% recurred >3 times. Conclusions: VKH in children is infrequent. It presents with optic-disc involvement and complications of posterior pole. It requires a high degree of suspicion, quick evaluation and early treatment, which include inmunosupressive and extended corticosteroid therapy. Nevertheless, a high rate of recurrence is seen among this group of patients. (AU)

Enfermedad de Vogt-Koyanagi-Harada y rehabilitación visual / Vogt-Koyanagi-Harada disease and visual rehabilitation

La enfermedad de Vogt-Koyanagi-Harada es una entidad infrecuente, multisistémica, de etiología desconocida, presuntamente autoinmune, caracterizada por panuveítis granulomatosa crónica bilateral y difusa, acompañada de participación tegumentaria, neurológica y auditiva, que afecta con mayor frecuencia la raza no caucasiana y, por lo general, a mujeres. Se presenta generalmente entre los 20-50 años de edad. Su incidencia varía geográficamente. Se estima que el 25 por ciento de los pacientes con esta enfermedad son ciegos legales; que el 25 por ciento puede presentar baja visión y el 50 por ciento agudeza visual mayor de 20/50. Se presenta una paciente de 50 años de edad con antecedentes de enfermedad de Vogt-Koyanagi-Harada, de 8 años de evolución, quien ha llevado tratamiento con antinflamatorios esteroideos sistémicos e inmunosupresores, así como terapia de apoyo con antinflamatorios esteroideos tópicos y ciclopléjicos. Acudió a la consulta de baja visión y se le realizó examen oftalmológico completo, estudios complementarios y se rehabilitó mediante el uso de ayudas ópticas y no ópticas para lograr el mayor aprovechamiento de su resto visual(AU)

Vogt-Koyanagi-Harada (VKH) is a rare, multisystemic, allegedly autoimmune disease of unknown etiology. It is characterized by chronic bilateral granulomatous and diffuse panuveitis, accompanied by tegumentary, neurological and hearing impairments that often affect the non-Caucasians and usually women. It usually occurs in the 20-25 years age group and its incidence varies with the geographic location. It is estimated that 25 percent of the patients with this disease are legally blind, the other 25 percent may have low vision and 50% present with visual acuity over 20/50. There is a 50 years-old patient with a history of Vogt - Koyanagi - Harada disease for 8 years. He had been treated with systemic steroid anti-inflammatory drugs and immunosuppressive therapy as well as supporting therapy with topical and cycloplegic steroid anti-inflammatories. The patient had gone to the low vision service looking for rehabilitation. He was performed a complete eye examination, supplementary studies and he was finally rehabilitated through the use of optical and non-optical aids in order to maximize his remaining vision(AU)

Indocyanine green angiography guided management of Vogt-Koyanagi-Harada disease

To report the management of Vogt-Koyanagi-Harada [VKH] disease based on indocyanine green angiography [ICGA]. VKH patients with acute episodes of inflammation [inaugural or recurrent] who had received standard ICGA-guided care were studied retrospectively. Standard of care included high dose systemic corticosteroids at presentation and close ICGA follow-up with addition of immunosuppressive agents and/or intensification of ongoing therapy when recurrent choroidal lesions were detected by ICGA. Visual acuity, number of subclinical recurrences, type and duration of therapy, proportion of quiescent patients after therapy, and ICGA findings were recorded. Nine patients including 8 female and one male subject were studied. Five patients had inaugural disease and 4 presented with recurrent acute episodes. Visual acuity increased from 0.86 +/- 0.36 to 1.14 +/- 0.34 in the right eyes, and from 0.77 +/- 0.34 to 1.05 +/- 0.33 in the left eyes. The number of ICGA-detected occult choroidal recurrences amounted to 13. Mean duration of treatment was 30.1 +/- 34.6 months leading to recurrence-free status after discontinuation of therapy in 6 cases with mean duration of 29.5 months. Continuous monitoring and aggressive therapy guided by ICGA in VKH disease prolongs treatment as compared to textbook guidelines but offers the prospect of reaching inflammation-free status after discontinuation of therapy. Zero tolerance to subclinical choroidal inflammation avoids irremediable evolution towards sunset glow fundus in patients treated early after the initial acute inflammatory attack

Vogt-Koyanagi-Harada disease occurring during pegylated interferon-alpha2b and ribavirin combination therapy for chronic hepatitis C / 대한간학회지

Vogt-Koyanagi-Harada (VKH) disease is a multisystem syndrome characterized by ocular (uveitis and retinal detachment), neurological (headache, tinnitus, and meningitis), and integumentary (vitiligo, alopecia, and poliosis) involvement. Although the pathogenesis of VKH disease is not well understood, an autoimmune T-cell response to a melanocyte-associated antigen is considered to be a cause of VKH disease. The complex immunological response to interferon and ribavirin may induce or exacerbate the autoimmune condition; however, VKH disease is a very rare complication associated with interferon therapy in chronic hepatitis C. We report a case of VKH disease occurring during pegylated interferon-alpha2b and ribavirin combination therapy for chronic hepatitis C.

Síndrome de Vogt-Koyanagi-Harada (SVKH): reporte de un caso / Vogt-Koyanagi-Harada (SVKH): case report

Introducción: Describimos un caso del síndrome de Vogt-Koyanagi-Harada (SVKH) de etiología desconocida. Caso Clínico: Mujer de 18 años de edad con antecedente de eritema conjuntival y disminución de la agudeza visual por seis meses, tratada con corticoides por presentar cefalea, trastorno de conciencia, crisis convulsiva y fiebre. Es admitida y se observa facies de luna llena, inyección conjuntival marcada, jiba cervical, poliosis incipiente, discreta alopecia. Reflejo corneal disminuido bilateralmente, signos meníngeos presentes, Babinski bilateral, uveítis exudativa anterior bilateral. Exámenes Auxiliares: leucocitosis con desviación izquierda, hiperglicemia, inmunoglobulinas séricas Ig G e Ig M positivas para Herpes 1y Toxoplasma, LCR: 27 células 100% mononucleares, 108 mg/dl de glucosa, 54mg/dl de proteínas. Discusión: El caso descrito presentó: fase prodrómica o meníngea, uveítica, de convalecencia y crónica - recurrente en el transcurso de la enfermedad. La evolución clínica fue favorable, pero la recurrencia luego del alta con disminución de la agudeza visual y uveítis bilateral orientó al diagnóstico de SVKH. Durante el seguimiento, la inflamación intraocular se mantuvo en forma crónica con pérdida considerable de la agudeza visual que no coincide con el pronóstico visual en el SVKH descrito en la literatura. Presentamos este caso por la inusual presentación y riqueza de síntomas.

Introduction: We describe a case of Vogt-Koyanagi-Harada syndrome (SVKH) of unknown etiology. Clinic case: 18 year-old female with a history of conjunctival erythema and decreased visual acuity from 6 months, treated with corticoids. She was admitted with headache, disturbance of consciousness and fever. At physical examination: full moon face, marked conjunctival injection, cervical jiba, incipient poliosis, and discrete alopecia. Decreased corneal reflex bilaterally. Meningeal signs, bilateral Babinski, anterior bilateral exudative uveitis. Laboratory tests: leukocytosis with left shift, hyperglycemia, serum immunoglobulins Ig G and Ig M positive for Herpes I and Toxoplasma, LCR: 27% mononuclear cells 100, 108 mg / dl glucose, 54mg/dl protein. Discussion: We describe a case that presented: meningeal or prodromal phase, uveítica, convalescent and chronic - recurrent in the course of their disease. The clinical outcome was favorable, but the recurrence after withdrawal of corticosteroids with decreased visual acuity and bilateral uveitis target in the diagnosis of SVKH. During follow-up intraocular inflammation remained in a chronic loss of visual acuity that does not match the visual prognosis in SVKH described in the literature. We present this case for the unusual presentation of symptoms wealth.

Síndrome de Vogt-Koyanagi-Harada e o seu acometimento multissistêmico / Vogt-Koyanagi-Harada's syndrome and its multisystem involvement

A síndrome de Vogt-Koyanagi-Harada é uma enfermidade rara que atinge os tecidos que contêm melanócitos, como os olhos, sistema nervoso central, orelha interna e pele. Alguns grupos étnicos têm maior propensão para desenvolver a doença, como os asiáticos, indianos e latino-americanos >e o gênero mais acometido é o feminino. O método realizado foi a revisão bibliográfica em livros, artigos publicados na internet e trabalhos disponíveis nos bancos de dados online MEDLINE, LILACS e SciELO. Foram selecionados os textos que abordassem a doença de forma abrangente, ressaltando suas manifestações otorrinolaringológicas. Revisão da literatura: A doença apresenta provável etiologia autoimune em que ocorre agressão à superfície dos melanócitos promovendo reação inflamatória com predomínio de linfócitos T. O alelo HLA DRB1*0405 é o mais associado à doença. As manifestações clínicas dividem-se em quatro estágios: prodrômico, uveítico, crônico e de recorrência. As manifestações otorrinolaringológicas ocorrem na fase uveítica e são caracterizadas por perda auditiva sensorioneural bilateral, zumbido e sintomas vestibulares. O diagnóstico é realizado através dos critérios diagnósticos da doença. O tratamento é realizado principalmente com corticoides. Considerações finais: É necessário que a síndrome de Vogt-Koyanagi-Harada seja uma doença reconhecida pelas diversas especialidades médicas, pois o atraso em seu diagnóstico pode resultar em sequelas oculares e cutâneas.

Vogt-Koyanagi-Harada's syndrome is a rare disease that affects tissues containing melanocytes such as eyes, central nervous system, inner ear and skin. Some ethnic groups have a higher probability of developing the disease like Asians, Indians and Latin Americans and most affected is the female gender. Methods: Literature was reviewed in books, articles published on the internet and papers available in the online databases MEDLINE, LILACS and SciELO. Texts were selected focusing on the otorhinolaryngological symptoms. Literature review: The disease probably has an autoimmune etiology in which aggression occurs on the surface of melanocytes by promoting inflammatory reaction with predominance of T lymphocytes. The HLA DRB1*0405 allele is the most associated with the disease. Clinical manifestations are divided into four stages: prodromal, uveitic, chronic and recurrent. The otorhinolaryngological symptoms occur at the uveitic stage characterized by bilateral sensorioneural hearing loss, tinnitus and vestibular symptoms. Diagnosis is made following diagnostic criteria of the disease. Treatment consists mainly of corticosteroids. Conclusions: Different specialities must recognize the importance of the Vogt-Koyanagi-Harada's syndrome.

New insights into Vogt-Koyanagi-Harada disease / Novos conhecimentos sobre a doença de Vogt-Koyanagi-Harada

Vogt-Koyanagi-Harada disease (VKH), a well-established multiorgan disorder affecting pigmented structures, is an autoimmune disorder of melanocyte proteins in genetically susceptible individuals. Several clinical and experimental data point to the importance of the effector role of CD4+ T cells and Th1 cytokines, the relevance of searching a target protein in the melanocyte, and the relevance of the HLA-DRB1*0405 in the pathogenesis of the disease. Vogt-Koyanagi-Harada disease has a benign course when early diagnosed and adequatey treated. Full-blown recurrences are rare after the acute stage of Vogt-Koyanagi-Harada disease is over. On the other hand, clinical findings, such as progressive tissue depigmentation (including sunset glow fundus) and uveitis recurrence, indicate that ocular inflammation may persist after the acute phase. Additionally, indocyanine green angiography findings suggest the presence of choroidal inflammation in eyes without clinically detectable inflammation. The aim of this paper is to review the latest research results on Vogt-Koyanagi-Harada disease pathogenesis and chronic/convalescent stages, which may help to better understand this potentially blinding disease and to improve its treatment.

A doença de Vogt-Koyanagi-Harada (VKH) afeta vários órgãos que têm em comum a presença de pigmento. É doença autoimune que agride os melanócitos de indivíduos geneticamente susceptíveis. Inúmeras evidências clínicas e experimentais demonstram a importância de células T CD4+ como células efetoras da resposta imune celular, das citocinas pró-inflamatórias Th1, da procura da proteína-alvo dentro do melanócito, e da relevância do HLA-classe II DRB1*0405 na patogênese desta doença. A doença de Vogt-Koyanagi-Harada apresenta bom prognóstico visual desde que o diagnóstico seja precoce e o tratamento instituído seja adequado. Recidivas com acometimento do segmento posterior são raras após a fase aguda da doença. No entanto, achados clínicos como a progressiva despigmentação do fundo, incluindo o aspecto em por do sol, e as recidivas da uveíte indicam que a inflamação ocular pode persistir mesmo após a fase aguda da doença. Os achados da angiografia com indocianina verde também sugerem a presença de inflamação da coróide mesmo em olhos sem inflamação clinicamente detectável. O objetivo do presente trabalho é rever os mais recentes estudos sobre a patogênese da doença Vogt-Koyanagi-Harada e sobre os aspectos clínicos da fase crônica e/ou convalescente da doença, permitindo melhores conhecimentos sobre esta doença potencialmente mórbida e oferecendo terapias mais adequadas.

Acute Lymphoblastic Leukemia Manifesting as Acute Vogt-Koyanagi-Harada Disease

We describe a case of bilateral exudative retinal detachment associated with prodromal symptoms simulating the presentation of acute Vogt-Koyanagi-Harada disease that was eventually diagnosed as acute lymphoblastic leukemia. A 42-year-old man presented with sudden visual loss in both eyes for two weeks. He complained of intermittent headache, neck stiffness and tinnitus for a month. His best-corrected visual acuities were 20/200 in both eyes. Fluorescein angiography, optical coherence topography and indocyanine green angiography featured bilateral serous retinal detachments. A clinical diagnosis of incomplete type Vogt-Koyanagi-Harada disease was considered. However, complete blood cell count showed a marked increase in the number of white blood cells and bone marrow examination revealed precursor B cell lymphoblastic leukemia. The patient started on induction chemotherapy. A week later, his best-corrected visual acuities were 20/25 and the serous retinal detachments were nearly absorbed in both eyes. Bilateral exudative retinal detachment associated with neurologic and auditory abnormalities may be a presenting sign of acute lymphoblastic leukemia. Clinicians should be aware of the possibility of leukemia in such patients.

Applicability of the 2001 revised diagnostic criteriain Brazilian Vogt-Koyanagi-Harada disease patients

PURPOSE: To determine the applicability of the international revised diagnostic criteria for Vogt-Koyanagi-Harada disease. METHODS: Retrospective study. Medical charts of 140 patients with the diagnosis of Vogt-Koyanagi-Harada disease, from the Uveitis Sector of the Federal University of Sao Paulo (UNIFESP), were revised and classified following the revised diagnostic criteria. RESULTS: Of the 140 patients, 12.85 percent fulfilled the criteria for complete disease, 29.28 percent incomplete disease, 28.57 percent "probable" Vogt-Koyanagi-Harada disease and 28.27 percent were considered not Vogt-Koyanagi-Harada disease. CONCLUSION: The authors consider that the international revised diagnostic criteria have good applicability and are very useful to help in the diagnosis of Vogt-Koyanagi-Harada disease.

OBJETIVO: Determinar a aplicabilidade do "Internacional revised diagnostic criteria for Vogt-Koyanagi-Harada disease". MÉTODOS: Estudo retrospectivo. Prontuários de 140 pacientes com diagnóstico de "Vogt-Koyanagi-Harada disease", do setor de uveítes da Universidade Federal de São Paulo (UNIFESP), foram revisados e classificados segundo o "Internacional revised diagnostic criteria". RESULTADOS: Dos 140 pacientes, 12,85 por cento preencheram os critérios para doença completa, 29,28 por cento doença incompleta, 28,57 por cento "provável" doença de Vogt-Koyanagi-Harada e 28,27 por cento foram consideradas como não sendo doença de Vogt-Koyanagi-Harada. CONCLUSÃO: Os autores consideraram que o "International revised diagnostic criteria" tem boa aplicabilidade e é muito útil no diagnóstico da doença de Vogt-Koyanagi-Harada.

Headache as a first manifestation of vogt-koyanagi-harada disease

To describe headache as an initial presentation of Vogt-Koyanagi-Harada [VKH] disease. Clinical Presentation and Intervention: A 56-year-old man initially presented with a 6-month duration of a continuous, diffuse headache of mild to moderate and sometimes of severe intensity. A CT of the brain was normal. Neurological examination was normal, including absence of symptoms and signs of meningismus. During the last 2 months, an intermittent eye pain, redness, and gradual loss of vision in both eyes was detected and subsequently he had tinnitus, malaise, nausea and mild meningismus. He was diagnosed as having VKH disease. Ophthalmologic examination revealed high intraocular pressure, requiring trabeculectomy with surgical iridectomy. The patient is now being treated with systemic steroids. VKH disease may initially present as sustained headache, without specific ophthalmologic symptoms and it should be considered in the differential diagnosis in patients with atypical but relentless headache

Vogt-Koyanagi-Harada syndrome in a 10-year-old child.

A 10-year-old female child attended outpatients department of Regional Institute of ophthalmology, Kolkata with the complaints of gradual dimness of vision of both eyes, recurrent pain, redness and photophobia. On examination, she was found to have bilateral anterior uveitis, poliosis and vitiligo. Laboratory tests were performed to rule out other causes of granulomatous uveitis. She was treated with topical and systemic steroids and topical cycloplegic. Then pars plana vitrectomy with lensectomy was carried out. The vision still remained poor.

Síndrome de Vogt-Koyanagi-Harada incompleta associada a HLA DRB1*01 em criança de quatro anos de idade: relato de caso / Incomplete Vogt-Koyanagi-Harada syndrome associated with HLA DRB1*01 in a 4-year-old child: case report

A síndrome de Vogt-Koyanagi-Harada é doença crônica, sistêmica e auto-imune, com manifestações oculares, nervosas, auditivas e tegumentares. Descrevemos aqui o caso de uma criança com início dos sintomas aos quatro anos e dois meses de idade, com positividade para o HLA DRB1*01.

Vogt-Koyanagi-Harada syndrome is chronic systemic autoimmune disease with ocular, nervous, auditory and tegumental manifestations. We report here the case of a child with onset of symptoms at four years and two months of age, with positive HLA DRB1*01.

Vogt-Koyanagi-Harada disease in Thai patients.

OBJECTIVES: To study the demographic, clinical manifestations, treatment and visual outcome of Vogt-Koyanagi-Harada disease (VKH) in a group of Thai patients. DESIGN: Retrospective case series. MATERIAL AND METHOD: Patients with VKH disease in Pramongkutklao Hospital from January 1998 to December 2002 were identified. Demographic data, clinical manifestations, treatment, and outcome were analyzed. RESULTS: Among the 33 patients, there were 12 males and 21 females with a mean age of onset of 35 +/- 13.4 year-old (range 17-67). Serous retinal detachment was detected in 48%. Transient hearing loss, vitiligo, poliosis and alopecia occurred in 18%, 24%, 15% and 33% respectively. Forty two percent of the patients were treated with systemic corticosteroids alone and 58% with a combination of steroids and immunosuppressive drugs. At the end of the follow-up, visual acuity (VA) improved in 55%, remained unchanged in 36% and worsened in 9% of the eyes. Final VA of equal or better than 20/40 was obtained in 76%. The mean follow-up time was 21.6 +/- 14.9 months (range 2-64). CONCLUSION: Demographic data, clinical manifestations, and treatment outcome of this group of Thai patients are comparable with studies from other countries.

Enfermedad de Vogt-Koyanagi-Harada: reporte de un caso y revisión de la literatura / Vogt-Koyanagi-Harada disease: report of a case and theme review

El propósito de este estudio es reportar un caso muy florido de enfermedad de Vogt-Koyanagi-Harada, y además hacer una revisión del tema, principalmente en cuanto a etiopatogenia, presentación clínica y criterios diagnósticos de la misma. En este caso, una paciente de 34 años de edad fue examinada por un cuadro de 1 semana de evolución de cefalea occipital, hipoacusia bilateral, y pérdida súbita de la agudeza visual en forma simétrica y bilateral, como principal queja. No había otros antecedentes patológicos o familiares de importancia. Dentro del abordaje de la paciente, se descartó causa alguna de hipertensión endocraneana mediante TAC, y los laboratorios generales fueron normales, excepto por una VES aumentada. Dentro de examen ocular, se encontraron hallazgos compatibles con proceso panuveítico bilateral, así como desprendimento de retina importante bilateral. El LCR reveleó pelocitosis de predominio linfocítico con hipoglucorraquia y leve hiperproteinorraquia, sin evidencia de microrganismos. La audiometría demostró un déficit neurosensorial simétrico, mientras la paciente se quejaba de sensación de inestabilidad y acúfeno izquierdo. Todos los hallazgos encontrados, nos orientaron muy fuertemente hacia el diagnóstico de Enfermedad de Vogt-Koyanagi-Harada, siendo éste caso muy florido y útil para una revisión del tema. Con el tratamiento médico correspondiente, la paciente evoluciono satisfactoriamente, egresando con una notable mejoría clínica, con tratamiento, para seguir su control ambulatoriamente.