Myelin oligodendrocyte glycoprotein antibody-associated optic neuritis: an update / Neurite óptica associada com anticorpo contra a glicoproteína oligondendrócita da mielina: uma breve atualização

ABSTRACT Myelin oligodendrocyte glycoprotein-immunoglobulin G (IgG)-associated optic neuritis has been established as a new entity of immune-mediated optic neuropathy. Patients usually present with recurrent optic neuritis, often bilaterally with initially severe vision loss and optic disc edema. However, in contrast to aquaporin 4-IgG-seropositive neuromyelitis optica spectrum disorder, visual recovery tends to be more favorable, with good response to steroid treatment. Another important differential diagnosis of myelin oligodendrocyte glycoprotein-IgG--associated optic neuritis is multiple sclerosis. Close monitoring for signs of relapse and long-term immunosuppression may be considered to maintain optimal visual function. The diagnosis can be made on the basis of the presence of a specific, usually serological, antibody against myelin oligodendrocyte glycoprotein (IgG; cell-based assay), and a demyelinating event (optic neuritis, myelitis, brainstem syndrome, or cortical lesions with seizures). The clinical spectrum of this newly recognized inflammatory demyelinating disease is expanding rapidly. We briefly review the epidemiological characteristics, clinical manifestations, diagnostic considerations, and treatment options of myelin oligodendrocyte glycoprotein-IgG-associated optic neuritis.

RESUMO A neurite óptica associada à glicoproteína de oligodendrócito de mielina-IgG foi estabelecida como uma nova entidade de neuropatia óptica imunomediada. Tipicamente os pacientes apresentam neurite óptica recorrente, muitas vezes bilateral, com perda de visão frequentemente severa e alta prevalência de edema do disco óptico na fase aguda. No entanto, em contraste com neuromyelitis optica spectrum disorder associada com presença de anticorpo contra aquaporina 4, a recuperação visual tende a ser mais favorável e responde bem ao tratamento com corticoide em altas doses. A esclerose múltipla representa outro importante diagnóstico diferencial de glicoproteína de oligodendrócito de mielina-IgG. O diagnóstico pode ser feito com base na presença de um anticorpo específico, geralmente sorológico contra glicoproteína de oligodendrócito de mielina (IgG, ensaio baseado em células), e presença de evento desmielinizante (neurite óptica, mielite, síndrome do tronco cerebral, lesões corticais com convulsões). O espectro clínico desta doença desmielinizante inflamatória recém-reconhecida está se expandindo rapidamente. Faremos uma breve revisão das características epidemiológicas, manifestações clínicas, considerações diagnósticas e opções de tratamento da neurite óptica associada à glicoproteína de oligodendrócito de mielina-IgG.

Neurite óptica secundária a infecção pelo Chikungunya Vírus / Optic neuritis secondary to chikungunya virus infection

RESUMO Apresentamos um caso de um paciente de 46 anos, sexo masculino com diagnóstico de neurite ótica em olho direito associado a infecção aguda por Chikungunya. Os sintomas iniciais eram dor e baixa acuidade visual em olho direito associado a febre e poliartralgia simétrica há uma semana. Ao exame a acuidade visual era de 20/60 em olho direito e 20/20 em olho esquerdo, fundoscopia evidenciou edema de papila à direita. Foi iniciado imediatamente pulsoterapia com metilprednisolona por 7 dias e foi observada melhora do quadro de neurite no seguimento de 1, 3 e 12 meses, porém melhora parcial da acuidade visual, Dentre as causas investigadas identificou-se sorologia anti Chikungunya IgM positivo.

ABSTRACT We present a case of optic neuritis secondary to Chikungunya virus infection. Male, 46 yo, initial symptoms were pain and low visual acuity in the right eye associated to fever and symmetrical polyarthralgia one week ago. At the examination the visual acuity was 20/60 in the right eye and 20/20 in the left eye, fundoscopy showed papillo edema on the right eye. Immediately initiated pulse therapy with methylprednisolone for 7 days and improvement of the neuritis was observed in the follow-up of 1, 3 and 12 months, but partial improvement of the visual acuity. Among the investigated causes, Chikungunya IgM positive serology was identified.

Optic neuritis and chorioretinitis as ocular manifestations of borreliosis in Brazil: three cases reported / Neurite óptica e coriorretinite como manifestações oculares da borreliose no Brasil: três casos relatados

Abstract Lyme disease is a systemic infection caused by a tick bite and transmission of the Borrelia burgdorferi spirochete. Species of tick vectors of the disease infest mainly wild or rural animals and rodents that may be asymptomatic reservoirs of the bacteria. Characteristic of the northern hemisphere, Lyme disease in Brazil takes on different characteristics, complicating diagnosis. This paper aims to describe three cases of Lyme-like disease in a city in the state of Bahia, Brazil, with ophthalmologic findings.

Resumo A doença de Lyme é uma infecção sistêmica causada pela picada do carrapato e transmissão da espiroqueta Borrelia burgdorferi. As espécies de carrapatos vetores da doença infestam, principalmente, animais silvestres, rurais e roedores que podem ser reservatórios assintomáticos da bactéria. Característica do hemisfério norte, a doença de Lyme no Brasil assume características distintas, dificultando seu diagnóstico. Esse trabalho tem por objetivo, descrever três casos da doença Lyme símile do Brasil, com achados oftalmológicos, em município do Estado da Bahia.

Optic neuritis complicating herpes zoster ophthalmicus in an immunocompetent patient / Zoster optic neuritis in immunocompentent

A 58-year-old woman presented with rash over the left side of the face and intense acute uveitis. Following careful review of the symptoms and dilated fundus examination unilateral optic neuritis was discovered. The rash was typical of varicella zoster dermatitis. Patients presenting with herpes zoster ophthalmicus should always undergo dilated fundus examination, as there is a potential risk of unexpected posterior segment inflammation. Early diagnosis and prompt treatment can avoid visual sequelae.

Paciente de 58 anos de idade apresentando erupção cutânea no lado esquerdo da face e intensa uveíte unilateral. Após cuidadosa revisão dos sintomas e exame de fundo do olho foi detectada neurite óptica. O rash era típico de dermatite por varicella zoster. Pacientes apresentando quadro de herpes zoster oftálmico devem ser submetidos ao exame de fundo do olho devido ao risco de inesperada inflamação do segmento posterior. Diagnóstico precoce e tratamento imediato podem evitar danos visuais.

A Case of Optic Neuritis Complicating Herpes Zoster Ophthalmicus in a Child

Here we report a case of optic neuritis in the setting of herpes zoster ophthalmicus (HZO) in a child. A six-year-old girl presented with HZO in the right eye. During the hospitalization, her visual acuity decreased. Fluorescein angiography (FAG) and optical coherence tomography revealed optic neuritis in the affected eye. Visual acuity improved with one month of treatment with acyclovir and steroids. FAG analysis showed no evidence of leakage at the optic disc. At one year post treatment, the patient's fundus exam and vision were normal. Therapy with antivirals and steroids may be effective in patients with childhood HZO optic neuritis

Visual function following treatment of optic neuritis

Optic neuritis is a common cause of visual loss in young adults and often the first manifestation of multiple sclerosis. Recent studies have shown that treatment with intravenous methylprednisolone results in more rapid recovery of vision, but without any long term difference in visual acuity. This study was carried out to evaluate clinical characteristics of patients with optic neuritis and visual outcome after intravenous methylprednisolone treatment. In a case series study, 40 cases with optic neuritis were evaluated. Before and after treatment with methylprednisolone according to optic neuritis treatment trial, visual acuity, contrast sensitivity, color vision, streopsis and visual field were analyzed. 67.5% of the patients were females. The most common age group was between 20 and 40 [60%]. Blind spot enlargement and other visual field defects were also returned to relatively normal value after the treatment. Central scotoma was the most common field defect [70%] and mild Dutan defect was the most common color vision [60%] defect in this study. Visual acuity, contrast sensitivity, color vision, streopsis and visual field were significantly reduced in optic neuritis, relatively returning to the normal level after treatment. It seems that the assessment of other visual functions, besides visual acuity, is important in a patient with optic neuritis, because patients usually remain aware of visual deficits other than decreased visual acuity

Bartonelosis ocular: Reporte de tres casos / Ocular bartonellosis: Report of three clinical cases

The characteristic clinical presentation of cat scratch disease is subacute regional lymphadenopathy; nevertheless, 5-25 percent of Bartonella henselcie infections may present an atypical or systemic form, with potential eye involvement. We describe three clinical cases of ocular bartonellosis in two adolescents and one young adult, who had close contact with cats; all of them presented persistent fever ranging from 15 to 21 days, and two of them developed a sudden unilateral loss of visual acuity associated with optic neuritis. The other patient presented retinal choroiditis and unilateral retinal microgranulomas, with normal visual acuity. Patients received macrolides as sole antimicrobial or in association with rifampin, and one patient was additionally treated with systemic corticoids. The outcome was favorable in two patients; one patient developed a permanent visual deficit. Ocular bartonellosis must be suspected in patients with close contact to cats or with cat scratches whom develop persistent fever and sudden loss of visual acuity.

La enfermedad por arañazo de gato se manifiesta típicamente como una linfadenopatía regional sub-aguda; sin embargo, 5 a 25 por ciento de los pacientes infectados por Bartonella henselae desarrollan formas atípicas o sistémicas de la enfermedad, pudiendo evolucionar con compromiso ocular. Consideramos de interés describir las características clínicas, tratamiento y evolución de tres pacientes con bartonelosis ocular, dos adolescentes y un adulto joven, que tenían antecedentes de contacto y/o rasguño por gatos. Todos cursaron con síndrome febril prolongado, con 15 a 21 días de duración, asociado a pérdida súbita de la agudeza visual unilateral en dos casos, cuya fondoscopia reveló neuritis óptica. El otro paciente presentó retino-coroiditis y microgranulomas retiñíanos, con agudeza visual conservada. Todos recibieron tratamiento antimicrobiano con macrólidos solos o asociados a rifam-picina y uno recibió además corticosteroides sisté-micos. La evolución fue satisfactoria en dos, quedando un paciente con déficit visual permanente. Recomendamos sospechar bartonelosis ocular en pacientes con antecedentes de contacto y/o rasguños por gatos, que cursan con un síndrome febril prolongado y/o disminución súbita de la agudeza visual.

Early onset optic neuritis following measles-rubella vaccination

To report two cases of optic neuritis with onset less than 24 hours following measles-rubella [MR] vaccination. Two teenage patients developed acute optic neuritis 6 to 7 hours after MR booster vaccination. The first patient demonstrated bilateral papillitis and severe visual loss but improved significantly with pulse intravenous steroid therapy with methylprednisolone 500 mg/day. The second patient had unilateral retrobulbar optic neuritis and demonstrated excellent visual recovery without intervention. Acute optic neuritis is a rare complication of MR vaccination and may occur early after immunization

Comparative evaluation of megadose methylprednisolone with dexamethasone for treatment of primary typical optic neuritis.

AIM: To compare the efficacy of intravenous methylprednisolone and intravenous dexamethasone on visual recovery and evaluate their side-effects for the treatment of optic neuritis. MATERIALS AND METHODS: Prospective, randomized case-controlled study including 21 patients of acute optic neuritis presenting within eight days of onset and with visual acuity less then 20/60 in the affected eye who were randomly divided into two groups. Group I received intravenous dexamethasone 200 mg once daily for three days and Group II received intravenous methylprednisolone 250 mg/six-hourly for three days followed by oral prednisolone for 11 days. Parameters tested were pupillary reactions, visual acuity, fundus findings, color vision, contrast sensitivity, Goldmann visual fields and biochemical investigations for all patients at presentation and follow-up. RESULTS: Both groups were age and sex-matched. LOGMAR visual acuity at presentation was 1.10 +/- 0.52 in Group I and 1.52 +/- 0.43 in Group II. On day 90 of steroid therapy, visual acuity improved to 0.28 +/- 0.33 in Group I and 0.36 +/- 0.41 in Group II ( P =0.59). At three months there was no statistically significant difference in the color vision, contrast sensitivity, stereoacuity, Goldman fields and the amplitude and latency of visually evoked response between the two groups. The concentration of vitamin C, glucose, sodium, potassium, urea and creatinine were within the reported normal limits. CONCLUSION: Intravenous dexamethasone is an effective treatment for optic neuritis. However, larger studies are required to establish it as a safe, inexpensive and effective modality for the treatment of optic neuritis.

Visual outcome after intravenous dexamethasone therapy for idiopathic optic neuritis in an Indian population: a clinical case series.

PURPOSE: To evaluate the clinical profile, response to dexamethasone treatment and visual function outcome in Indian patients with acute optic neuritis. MATERIALS AND METHODS: We conducted an observational study of patients with acute optic neuritis who were treated with intravenous dexamethasone (100 mg in 250 ml of 5% dextrose over 1-2 hours daily, for three consecutive days) and had completed at least two years of follow-up. Parameters assessed included visual acuity, contrast sensitivity, color vision, visual fields, relative afferent pupillary defect (RAPD) and visually evoked potentials. Out of 40 patients studied, 26 patients (33 eyes) had all visual function parameters assessed. Twenty three patients (28 eyes) had completed two years of follow-up and were included for statistical analysis. RESULTS: Improvement in visual acuity was statistically significant for distance after 24 hours of the first dose (P = < 0.001) and for near vision after 24 hours of the second dose (P = 0.006); improvement in color and contrast sensitivity was statistically significant 24 hours after the third dose (P = < 0.001 for color vision and P = 0.013 for contrast sensitivity). Significant improvement in RAPD and visual fields were seen by 1 month (P = 0.005). Recurrence was seen in 4 eyes of 4 patients. No serious side effects were observed. At two years, 82.14% (23 out of 28) eyes had visual acuity > 20/40. CONCLUSION: Treatment with intravenous pulsed dexamethasone led to rapid recovery of vision in acute optic neuritis, without any serious side effects.

Neuropatia óptica auto-imune: relato de caso / Autoimmune optic neuropathy: case report

Descrevemos uma paciente de 9 anos, sexo feminino, com perda visual bilateral grave tratada com corticóide por via oral apresentando melhora em apenas um olho. Nove anos depois apresentou recidiva que inicialmente respondeu à pulsoterapia corticóide mas foi seguida de perda progressiva e completa da visão após a redução do tratamento. Novas tentativas terapêuticas não melhoraram a visão. Avaliação clínico-laboratorial não revelou doença sistêmica, mas apresentava anticorpos antinucleares (1/640), anti-Ro e anti-La positivos. Neuropatia óptica auto-imune é uma afecção rara, que simula neurite óptica idiopática e se caracteriza por perda visual aguda, sem doença sistêmica, mas com evidências laboratoriais de doença auto-imune, em especial o FAN positivo. Biópsia de pele freqüentemente mostra evidências de vasculite. Esta condição deve ser tratada agressivamente, com corticóide e imunossupressores, uma vez que o acometimento visual geralmente é mais grave do que o da neurite óptica idiopática/desmielinizante e pode ser irreversível.

We report on a 9-year-old female patient who had bilateral severe visual loss and was treated with oral corticosteroids. Visual improvement occurred in one eye. Nine years later she presented relapse of visual loss in her only seeing eye. Pulse corticosteroid therapy resulted in dramatic visual improvement followed, however, by progressive and complete visual loss as soon as the corticosteroid was tapered. Repeat treatment did not result in visual improvement. Clinical and laboratory investigation failed to find a systemic disease but the patient had positive antinuclear (1/640), anti-Ro and anti-La antibodies. Autoimmune optic neuropathy is a rare condition that may mimic an idiopathic optic neuritis and is characterized by acute visual loss, without systemic disease but with laboratory evidence of an autoimmune disorder, usually a positive ANA. A skin biopsy usually shows evidence of vasculitis. This condition should be treated aggressively, with corticosteroids and immunosuppressant, since the visual involvement is usually worse than that of idiopathic/desmyelinating optic neuritis.

Neuritis óptica por Bartonella Henselae: dos casos: Nº 1 / Optic neuritis by Bartonella Henselae: two cases: Nº 1

La neuritis ótica (NO) corresponde a una neuropatía óptica determinada por un proceso inflamatorio focal asociado a desmielinización [1]. Su etiología es variada. En niños es principalmente de naturaleza infecciosa o parainfecciosa lo que determina un curso generalmente benigno, sin embargo, al estar asociada a esclerosis múltiple (EM), donde corresponde a la primera manifestación en el 75 por ciento de los casos, adopta una connotación distinta dado el desalentador pronóstico que implica [2].

Neuritis óptica por Bartonella Henselae: dos casos: Nº2 / Optic neuritis by Bartonella Henselae: two cases: Nº2

La neuritis óptica es una patología infrecuente en la infancia, pudiendo presentarse de manera primaria o postinfecciosa y, evolutivamente, corresponder a la forma de inicio de una esclerosis múltiple. Sus manifestaciones clínicas incluyen la disminución progresiva de la agudeza visual y la alteración de la percepción de los colores y del campo visual. Sus causas son múltiples, y entre éstas se pueden destacar las patologías desmielinizantes, las vasculitis y las infecciosas. Se muestra el caso de una paciente de 8 años de edad que presentó neuritis óptica bilateral secundaria a la infección por Batonella henselae y se explican la forma de presentación, diagnóstico, tratamiento y evolución de esta patología.

Neurocisticercosis parenquimatosa activa: reporte de un caso y revisión de la literatura / Active parenchymatous neurocysticercosis: report of a case and review of literature

Se presenta el caso de una paciente nicaragüense con neurocisticercosis parenquimatosa activa, cuya manifestación principal es la pérdida de la agudeza visual. En los estudios de imágenes, por medio de Tomografía Axial Computarizada y Resonancia Magnética Nuclear, se observan lesiones sugestivas de toxoplasmosis o de cisticercosis. El diagnóstico se confirma al efectuar la determinación de anticuerpos anticisticerco en líquido cefalorraquídeo por medio de un ELISA; otras pruebas de laboratorio, como el análisis citoquímico de líquido cefalorraquídeo, hemograma y bioquímica en sangre, dieron resultados dentro de límites normales. Se le administra albendazone (500 miligramos) dos veces por día por 30 días acompañado de antiinflamatorios y anticonvulsivos. La agudeza visual no mejoró posterior al tratamiento. Palabras clave: Cystercus cellulosae, neurocisticercosis, Taenia solium, abendazole, neuritis óptica

Bisected macula following retrobulbar steroid injection.

A case of bilateral accidental globe penetration during administration of retrobulbar steroid for bilateral optic neuritis is discussed. One eye with bisected macula was managed successfully by vitrectomy, internal gas tamponade, and postoperative laser to the edges of the retinal tear. The fellow eye was blind due to central retinal artery occlusion.

Mega dose intravenous methylprednisolone in bilateral simultaneous optic neuritis.

OBJECTIVES: The study was carried out to assess the efficacy of megadose intravenous methylprednisolone in patients of bilateral simultaneous optic neuropathy (BSON) of unknown etiology. METHODS: Fifteen consecutive patients admitted in neurology unit were included in the study. These patients were subjected to various investigations including MRI and CSF exam to exclude all known causes of optic neuropathy including multiple sclerosis. All patients were put on single dose intravenous methylprednisolone, 1 gm/day in adults and 500 mg/day in children for three days. Pre and post-treatment visual parameters including visual evoked potentials (VEP) were carried out. RESULTS: There was a female preponderance and the average age was 28 years. The visual acuity and P100 latences of visual evoked potentials (VEP) improved in all cases. The improvement was statistically significant (P < 0.001). CONCLUSION: Use of intravenous methylprednisolone is a preferred drug in bilateral simultaneous optic neuropathy (BSON) as compared to oral or retrobulbar steroids.

Neuritis retrobulbar inducida por amiodarona: caso clínico / Amiodarone induced retrobulbar neuritis: report of 1 case

We report a 66 years old male, with an ophtalmologic history of long sightedness, admitted to the hospital due to paroxysmal atrial fibrillation crises in the context of a coronary heart disease. He was treated with iv amiodarone, receiving a total dose of 6 g in 72 hours. After the third day of treatment, the patient noticed a correction of his long sightedness and 24 h later, he complained of blurred vision and orbital frontal headache. Visual field examination revealed a concentric retraction of visual field and a centrocecal scotoma in both eyes. Amiodarone was withdrawn and dexametasone treatment was begun. Three days after amiodarone discontinuation, sight improved and visual field returned to normal. Although retrobulbar neuritis has been associated to various drugs, amiodarone has not been considered as a possible agent

Neuropatía epidémica forma óptica. Ensayo terapeútico con vitaminas e interferones (alfa 2b recombinante y alfa natural) / Optic neuritis epidemic. Therapeutic trial with vitamins and interferons (recombinant alpa-2b and natural alpha)

Con el propósito de evaluar la eficacia, tolerancia y relación riesgo-beneficio de las vitaminas e interferones, se ejecutó un ensayo terpeutico multicéntrico, controlado y sin enmascaramiento, en 108 pacientes ingresados en 4 hospitales de Ciudad de La Habana con el diagnóstico de neuropatía epidémica forma óptica (NOE), durante el primer trimestre de 1992. Los pacientes se distribuyeron en 3 grupos con los siguientes tratamientos : interferón (INF) alfaleucocitario, interferón alfa 2b recombinante, que se asociaron a un esquema de vitaminas A,E, complejo B y ácido fólico, y se diseño un grupo control al que se le administró sólo este esquema. Los pacientes fueron hospitalizados y evaluados al ingreso, al alta hospitalaria (21 días de tratamiento), al mes y a los 3 meses del alta. La eficacia fue determinada siguiendo criterios de mejoría y recuperación, según una valoración oftalmológica integral de los pacientes y se ccalculó la ganancia de la agudeza visual. La tolerancia se estimó monitoreando la aparción de alguna reacción adversa medicamentosa (RAM) durante 21 días

La ozonoterapia en la neuropatía epidémica forma óptica ¿Beneficio y riesgo? / Ozone therapy in the management of the optic neuritis epidemic. Benefits od risks?

Se realizó un ensayo clínico terapéutico multicéntrico en Ciudad de La Habana, en 576 pacientes afectados de neuropatía epidémica forma óptica, en el período comprendido entre abril de 1993 y febrero de 1994. Se emplearon 11 esquemas de tratamiento, distribuidos aleatoriamente entre los hospitales participantes. Los grupos de tratamiento incluidos fueron : dexametasona, metilprednisolona, hidroxicobalamina+metionina+tiosulfato de sodio, vitaminas, magnetoterapia, ozonoterapia, oxigenación hiperbárica, electroforesis endonasal con vitamina B1, factor de trasnferencia, interferón (INF) alfa natural e interferón alfa 2b recombinante. A todos los pacientes del estudio se les administró vitaminas de base y se consideró a aquéllos tratados con vitaminas sólo como grupo control. La evaluación de los casos se realizó a los 21 días (alta hospitalaria), al mes, 3 y 6 meses, respectivamente. El tratamiento con ozono reflejó diferencias estadísticamente significativas (p<0,05) (en cada uno de los cortes evaluativos efectuados) en cuanto a mejoría y recuperación de los casos tratados con ese proceder. En el resto de los esquemas terapéuticos empleados no se encuentran diferencias significativas. Se evidenció la utilización de las vitaminas