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.

El desafío de la neuropatía óptica epidémica cubana / The challenge of the Cuban epidemic optic neuropathy

A partir del trienio 1989-1991 la economía cubana comenzó a debilitarse por la caída de los precios del azúcar y el petróleo, así como por el descenso de la cotización del dólar americano y la desintegración de la Unión Soviética, que trajo aparejada la carencia de divisas convertibles, combustibles, alimentos, y además imposibilitó la solicitud de créditos a instituciones financieras internacionales. Se inició, así, el llamado «período especial¼, fundamentado en un plan para enfrentar un posible bloqueo militar en tiempos de paz, y durante el cual fueron racionalizados estrictamente los alimentos y disminuyeron las industrias esenciales y el transporte. En tales circunstancias apareció una rara enfermedad que se convirtió en epidemia, la cual fue nombrada neuropatía óptica epidémica cubana; esta afectó a más de 50 000 cubanos y se convirtió en un verdadero desafío para la comunidad médica del país. Al respecto, en el presente artículo se analizan sucesos históricos relacionados con el surgimiento de dicha oftalmopatía, se defiende la teoría de su causa tóxico-nutricional debido a la situación de Cuba en aquel momento y se destaca el liderazgo del Comandante en Jefe Fidel en la conducción de las acciones multidisciplinarias que llevaron a controlar dicha epidemia, lo que resultó un logro para el Sistema Nacional de Salud y un triunfo de la Revolución cubana.

Since the triennium 1989-1991 the Cuban economy began to weaken due to the fall of sugar and petroleum prices, as well as due to the descent in the rate of the American dollar and due to the disintegration of the Soviet Union that brought the lack of convertible foreign currencies, fuels, foods, and disabled the application of credits to international financial institutions. This way, began the so call "special period", based in a plan to face a possible military blockade in times of peace, and during which foods were strictly rationalized and the essential industries and transport diminished. In such circumstances a strange disease appeared that became epidemic, which was named Cuban epidemic optic neuropathy; it affected more than 50 000 Cubans and became a true challenge for the medical community of the country. In this respect, historical events related to the emergence of this ophtalmopathy are analyzed in this work, that defends the theory of its toxic-nutritional cause due to the situation of Cuba in that moment and the leadership of Commander in Chief Fidel is outstanding in the conduction of the multidisciplinary actions that lead to control this epidemic, what became an achievement for the Health National System and a victory of the Cuban Revolution.

Neuritis óptica pediátrica: un desafío diagnóstico / Pediatric optic neuritis: a diagnostic challenge Pediatric optic neuritis: a diagnostic challenge

Introducción: la neuritis óptica requiere un diagnóstico y manejo desafiante, se puede presentar de forma aislada o asociada con trastornos inflamatorios, siendo hasta 25% de la clínica de los síndromes desmielinizantes agudos pediátricos. La incidencia anual estimada de neuritis óptica pediátrica es 0.2 por 100.000 niños, con una preponderancia femenina y una edad media de presentación de 9 a 11 años. Presentación del caso: paciente de 12 años con cuadro agudo de diplopía, dolor ocular izquierdo sin antecedentes relevantes, en quien se encontró parálisis del VI par izquierdo y resonancia magnética cerebral (RMc) con realce en nervio óptico, iniciándose tratamiento con corticoterapia endovenosa, previo descarte de patologías infecciosas, con evolución satisfactoria. Conclusiones: en el espectro de la neuritis óptica es importante el conocimiento de sus diferentes etiologías, debido a que el tratamiento y pronóstico dependen de la causa.

Introduction: optic neuritis (ON) requires a challenging diagnosis and management. It can appear as an isolated condition or in association with inflammatory disorders, being 25% of the clinical manifestations of pediatric acute demyelinating syndromes. The estimated annual incidence of pediatric ON is of 0.2 per 100.000, with a female preponderance and a mean age at onset of 9 to 11 years. Case report: a 12-year-old patient presenting with acute diplopia and left ocular pain, referring no relevant past history. Left sixth nerve palsy and brain magnetic resonance imaging (MRI) showing optic nerve enhancement, were evidenced. Therapy with intravenous corticosteroids was started, once an infectious etiology had been ruled out, with satisfactory progression. Conclusions: in the optic neuritis spectrum disorders, knowledge regarding other potential etiologies is key to treatment and prognosis, which depend on the cause.

Perfil clínico y epidemiológico de los pacientes con diagnóstico de neuromielitis óptica / Clinical and epidemiological profile of patients with neuromyelitis optica diagnosis

INTRODUCCIÓN. El trastorno del espectro de neuromielitis óptica, enfermedad inflamatoria, desmielinizante, afecta al sistema nervioso central, frecuente en poblaciones no caucásicas como la ecuatoriana. El retraso en su diagnóstico y tratamiento provoca discapacidad que se puede prevenir. OBJETIVO. Determinar el perfil clínico y epidemiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. MATERIALES Y MÉTODOS. Estudio descriptivo transversal. Población de 45 Historias Clínicas y una muestra de 41 de pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica atendidos en la Unidad de Neurología del Hospital de Especialida-des Carlos Andrade Marín, período enero 2005 a diciembre 2019. Se realizó análisis univarial. Se aplicó el programa estadístico International Business Machines Statistical Package for the Social Sciences, versión 25. RESULTADOS. El 76,0% (31; 41) fueron mujeres. Datos promedios: edad 48,9 años; diagnóstico definitivo demoró 4,12 años, desde el inicio de los síntomas; tiempo de diagnóstico fue 3,17 años; 3,7 brotes en total; el 87,8% (36; 41) con un fenotipo recurrente. La media de duración de la enfermedad fue de 6,8 años. En el 70,7% (29; 41), se identificaron anticuerpos anti-AQP4 en suero mediante inmunofluorescencia directa, el 51,2% requirieron para la marcha apoyo uni o bilateral. El 43,9% (18; 41) debutó con neuritis óptica; el 31,7% (13; 41) presentaron mielitis como primer síntoma y el 24,4% (10; 41) la combinación de neuritis óptica y mielitis fueron los síntomas iniciales. CONCLUSIÓN. Se determinó el perfil clínico y epi-demiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. Existió demora en el diagnóstico definitivo de los pacientes desde el inicio de los síntomas, lo que se tradujo en un aumento de la discapacidad.

INTRODUCTION. Neuromyelitis optica spectrum disorder, an inflammatory, demyelinating disease, affects the central nervous system, common in non-Caucasian popu-lations such as Ecuadorians. The delay in its diagnosis and treatment causes disabi-lity that can be prevented. OBJECTIVE. To determine the clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder. MATERIALS AND METHODS. Cross-sectional descriptive study. Population of 45 Medical Records and a sample of 41 patients with a diagnosis of neuromyelitis optica spectrum disor-der seen at the Neurology Unit of the Carlos Andrade Marín Specialties Hospital, period from January 2005 to December 2019. Univariate analysis was performed. The statistical program International Business Machines Statistical Package for the Social Sciences, version 25 was used. RESULTS. 76,0% (31; 41) were women. Average data: age 48,9 years; definitive diagnosis took 4,12 years from the onset of symptoms; time to diagnosis was 3,17 years; 3,7 outbreaks in total; 87,8% (36; 41) with a recurrent phenotype. The average disease duration was 6,8 years. In 70,7% (29; 41), anti-AQP4 antibodies were identified in serum by direct immunofluorescence, 51,2% required uni- or bilateral su-pport for walking. Optic neuritis started in 43,9% (18; 41); 31,7% (13; 41) had myelitis as the first symptom and 24,4% (10; 41) the combination of optic neuritis and myelitis were the initial symptoms. CONCLUSION. The clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder was determined. There was delay in the conclusive diagnosis of patients from the beginning of symptoms, which resulted in increased disability.

Uncommon progression of toxoplasmic papillitis: patient perception and case report / Papilite por toxoplasmose ocular com evolução incomum: percepção do paciente e relato de caso

ABSTRACT Ocular toxoplasmosis frequently presents as necrotizing retinochoroiditis and, less often, as peripapillary chorioretinitis and/or papillitis. The progression from papillitis to peripapillary retinochoroiditis has been rarely described. We report the case of a 52-year-old patient living in southern Brazil, who developed papillitis in the right eye and was treated with systemic corticosteroids (prednisone 0.6 mg/kg/day and pulse therapy with methylprednisolone 15 mg/kg/day, for 3 days). After 14 days, the patient developed peripapillary retinochoroiditis with vitritis and decreased visual acuity (20/60), and was immediately initiated on the classic oral treatment for toxoplasmosis, consisting of pyrimethamine (50 mg/day), sulfadiazine (4 g/day), folinic acid (15 mg every 3 days) and prednisone (0.6 mg/kg/day). The visual acuity of the right eye normalized after treatment (20/20), which lasted approximately 70 days, but scotomas were detected on visual field examination, especially in the lower nasal quadrant. Although two studies mentioned this presentation, our report emphasizes the possible manifestation of ocular toxoplasmosis as papillitis in the initial phase, with progression to peripapillary retinochoroiditis and permanent visual field defects, which justifies early treatment for toxoplasmosis in suspected cases, especially in endemic regions.

RESUMO A toxoplasmose ocular manifesta-se com maior frequência por um quadro de retinocoroidite necrotizante e, com menor frequência, por coriorretinite justapapilar e/ou papilite. A evolução de papilite para retinocoroidite justapapilar raramente foi descrita. Apresenta-se o relato de caso de uma paciente de 52 anos, habitante da Região Sul do Brasil, que iniciou com quadro de papilite em olho direito, sendo tratada com corticoides sistêmicos (prednisona 0,6/mg/kg ao dia e pulsoterapia com metilprednisolona 15mg/kg ao dia, por 3 dias), mas, após 14 dias, evoluiu para retinocoroidite justapapilar, com vitreíte e diminuição de acuidade visual (20/60), sendo imediatamente instituído o tratamento via oral clássico para toxoplasmose, com pirimetamina (50 mg ao dia), sulfadiazina (4 g ao dia) e ácido folínico (15 mg a cada 3 dias), e mantida a prednisona (0.6 mg/kg/dia). A acuidade visual do olho direito normalizou após o tratamento (20/20), que durou em torno de 70 dias, porém desenvolveu escotomas ao exame de campo visual, sobretudo de quadrante nasal inferior. Embora tenham sido encontrados dois trabalhos que mencionam essa forma de apresentação, o presente relato destaca-se por enfatizar a possibilidade de manifestação da toxoplasmose ocular por meio de papilite na fase inicial, que evolui com retinocoroidite justapapilar, causando defeito permanente de campo visual, justificando que se avalie a instituição de tratamento precoce para toxoplasmose dos casos suspeitos, sobretudo em região endêmica.

Perdas visuais agudas: uma revisão dos últimos 10 anos de causas não infecciosas e não maculares / Acute visual loss: a review from the last 10 years of non-infectious and non-macular causes

Objetivo: o conhecimento das principais causas de perda visual aguda possibilita o diagnóstico precoce do paciente, o que favorece o tratamento mais rápido e eficaz, a fim de preservar a acuidade visual. Neste artigo de revisão, as principais causas não infeciosas e não oriundas da mácula são abordadas, a fim de buscar e revisar os tópicos mais pertinentes sobre cada tema, como as manifestações clínicas e os tratamentos mais utilizados. Métodos: trata-se de uma revisão de literatura, com 30 artigos selecionados e revisados da plataforma MEDLINE. Resultados: as causas de perda visual aguda discutidas são cinco. Primeiro, as por baixa acuidade visual, súbita, unilateral, sem dor e sem hiperemia: hemorragia vítrea, descolamento de retina, oclusão vascular de retina e neuropatia óptica isquêmica anterior. Posteriormente, por baixa acuidade visual, súbita, unilateral, com dor e sem hiperemia: neurite óptica. Por meio deste estudo, alguns fatores de risco podem ser evidenciados e os principais tratamentos destacados. Conclusão: o diagnóstico precoce das perdas visuais agudas deve ser realizado, com os exames físicos adequados, como a fundoscopia e os exames complementares necessários solicitados. Além disso, o encaminhamento ao oftalmologista é de extrema importância para minimizar sequelas e evitar complicações.

Objective: The knowledge of the main causes of acute visual loss enables the early diagnosis of the patient, which favors faster and more effective treatment in order to preserve visual acuity. In this review article, the main non-infectious causes not originating from the macula are addressed in order to search and review the most relevant topics on each theme, such as the clinical manifestations and the most used treatments. Methods: This is a literature review with 30 articles selected and reviewed from the MEDLINE platform. Results: Five causes of acute visual loss are discussed. First, those for sudden, unilateral, without pain, and without hyperemia low visual acuity are reviewed: vitreous hemorrhage, retinal detachment, retinal vascular occlusion, and anterior ischemic optic neuropathy. Subsequently, one due to low visual acuity, sudden, unilateral, with pain and without hyperemia is evaluated: optic neuritis. Through this study, some risk factors and main treatments can be highlighted. Conclusion: The early diagnosis of acute visual loss should be performed with appropriate physical exams, such as fundoscopy and the necessary complementary exams. In addition, referral to an ophthalmologist is extremely important to minimize sequelae and avoid complications.

Acute disseminated encephalomyelitis (ADEM) associated with mosquito-borne diseases: Chikungunya virus X yellow fever immunization

Abstract Acute disseminated encephalomyelitis (ADEM) is a demyelinating autoimmune neuropathic condition characterized by extensive bilateral and confluent lesions in the cerebral white matter and cerebellum. The basal ganglia and gray matter may also be involved. In most cases, the symptoms are preceded by viral infection or vaccination. In this report, we present a case of ADEM associated with optic neuritis presenting alongside two potential triggering factors: chikungunya virus infection and yellow fever immunization.

Meningoencephalitis and optical neuritis caused by CRYPTOCOCCUS GATTII in an immunocompetent patient

The following case is of a 59-year-old man, undergoing no medication, with no pathological history or others risk factors, who presented dizziness, fever and asthenia twenty days before admission. The patient was admitted for investigation when the asthenia intensified, followed by seizures. On admission, blood count, biochemical tests and chest computed tomography were normal, a serological test for anti-HIV proved negative, while the magnetic resonance of the brain showed signs suggestive of meningoencephalitis. Cerebrospinal fluid (CSF) analysis suggested bacterial meningitis due to increased leukocytes with a predominance of polymorphonuclear cells, reduced glucose and increased proteins as well as positive Gram cocci in pairs by Gram and negative fungi by India ink test. Treatment with ceftriaxone was started. Since there was no significant improvement, CSF analysis was repeated on the seventh day of treatment. Intracranial pressure was measured by manometry (29 mmHg) and CSF analysis showed the presence of encapsulated yeasts similar to Cryptococcus neoformans by the India ink test. The treatment was modified to liposomal amphotericin B and flucytosine; the intracranial hypertension was controlled by repeated CSF punctures. After fourteen days of antifungal treatment, the patient presented visual turbidity and bilateral papillar edema, so corticosteroid therapy was prescribed. The evolution was favorable, with progressive resolution of symptoms, improvement of CSF parameters and visual acuity. The patient was discharged eight weeks after admission, with outpatient guidance. Corticosteroid therapy associated with antifungal therapy proved to be beneficial in this case, since following the introduction of corticosteroids there was progressive visual improvement.

Neuritis óptica recurrente posterior a encefalomielitis diseminada aguda en pediatría. Reporte de un caso / Recurrent optic neuritis after acute disseminated encephalomyelitis in pediatrics. A case report

Abstract: Optic Neuritis (ON) is a condition caused by the inflammation of the optic nerve, causing diminished visual acuity and ocular pain. It is tightly related to Multiple Sclerosis (MS), often being the first demyelinating event. There is a 31% risk of recurrence during the first 10 years after the diagnosis, and 48% of the patients end up being diagnosed with Multiple Sclerosis1. Imaging studies like brain MRI (Magnetic Resonance Imaging) have a critical role in the diagnosis and prognosis of ON, as well as in the recognition of MS.2 The patient is a 11-year-old girl with recurrent ON and past medical history of Acute Disseminated Encephalomyelitis (ADEM) when she was 5 years old. ON diagnosis was based on clinical findings as well as on ophtalmologic, electrophysiologic and imaging studies. The recurrent episodes of ON improved after the use of high dose steroids. Recurrences were observed after titration of the dose, but remission was achieved after adjustment of treatment. During one of the recurrent episodes, blood work was performed to evaluate possible underlying infectious, demyelinating or autoinmmune process. Anti-MOG antibodies were found positive. The patient at the moment is not presenting with any other criteria suggesting MS or Optic Neuromyelitis, but long term follow up is adviced. Key words: Optic neuritis (ON), multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), magnetic resonance imaging (MRI), cerebrospinal fluid (CSF), right eye (RE); visual acuity (VA), optic neuromyelitis (ONM)

Clinical and electrophysiological correlation in patient with optic neuritis

A descriptive and traverse study was performed in patients with diagnosis of optic neuritis, that attended to the neuro ophthalmology service of the ophthalmological center, at the Santiago General Hospital Dr. Juan Bruno Zayas Alfonso , from the province of Santiago de Cuba, with less than a week of evolution of the disease, and visual acuity greater than 0.3, cooperatives and without refractive opacities, or neuro ophthalmologic diseases, during the period from December 2017 to November 2018. Ophthalmological clinical evaluations and electrophysiological studies of visual evoked potentials to pattern reversal, at diagnosis and eight weeks of evolution were carried out. Objective: To identify the relationship possible between the electrophysiological parameters obtained by visual evoked potentials with the ophthalmological clinical evaluation. Results: alterations predominantly of the parameters of latency and duration of the visual evoked potentials, in relation to the impairment of the clinical evaluations explored. Conclusions: The visual evoked potentials are an effective tool in diagnosis, and complement the clinical evaluation of patients with optic neuritis(AU)

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.

Neuropatia óptica tóxica por inalação de metanol / Optic neuropathy toxic after methanol inhalation

Resumo A intoxicação por metanol é um evento considerado raro, principalmente quando feita por via inalatória. Retratou-se neste presente estudo um relato de caso de um paciente que desenvolveu neurite óptica tóxica após exposição ao metanol e ácido acético por via inalatória em seu ambiente de trabalho. Foi descrito sobre as terapias obtidas na literatura, bem como as manifestações clínicas e o manejo a este paciente.

Abstract The methanol toxicity is considered rare event , especially when taken by inhalation . It was portrayed in the present study a case report of a patient who developed toxic optic neuritis after exposure to methanol and acetic acid by inhalation in the workplace . It was described for the therapies from the literature as well as the clinical manifestations and management in this patient.

Recurrent idiopathic neuroretinitis as a spectrum of atypical optic neuritis: a case report and literature review / Neurorretinite recorrente idiopática como espectro da neurite óptica atípica: relato de caso e revisão da literatura

ABSTRACT Recurrent idiopathic neuroretinitis is an inflammatory optic neuropathy characterized by optic nerve edema and macular star, associated with painless and recurrent episodes of visual loss, poor visual prognosis, and visual field defects related to nerve fiber layer injury. The disorder is sometimes mistaken for atypical optic neuritis. However, early diagnosis is important for visual recovery. Long-term immunosuppression has been shown to reduce the rate of recurrence and protect against severe and irreversible vision loss.

RESUMO Neurorretinite recorrente idiopática é uma neuropatia óptica inflamatória caracterizada por edema do nervo óptico e estrela macular associada a episódios recorrentes de perda visual indolor, baixo prognóstico visual e desfeitos de campo visual relacionados a injúria da camada de fibras nervosas. Essa condição pode ser confundida com neurite óptica atípica e seu correto diagnóstico é importante para o prognóstico visual, uma vez que a imunossupressão continua previne episódios recorrentes que podem levar a perda visual severa e irreversível.

Neuritis óptica en presencia de anticuerpos antifosfolipidos de origen sifilítico: un diagnostico olvidado / Optic neuritis in the presence of antiphospholipid antibodies of syphilitic origin: a forgotten diagnosis

Resumen: la neuritis óptica es infrecuente como manifestación de sífilis ocular, la falta de características típicas genera retraso en el diagnóstico. Describimos el caso de una mujer de 47 años , inmunocompetente con historia de 4 meses de disminución progresiva de la agudeza visual de predominio izquierdo, asociado a dolor, inyección conjuntival y cefalea, al examen físico con visión de bultos y al fondo de ojo con signos de inflamación ocular dentro de la evaluación diagnostica presenta: VDRL y FTA-ABS positivo en suero, positividad de ANAS y anticuerpos anticardiolipinas igG, LCR con VDRL reactivo, se diagnostica neuritis óptica por neurosifilis en presencia de anticuerpos antifosfolipidos, iniciando tratamiento con penicilina cristalina 24 000 000 de unidades día por 14 días. En pacientes con signos de inflamación ocular debe realizarse VDRL, confirmarse con prueba treponemica, y realizar punción lumbar, el tratamiento precoz se asocia a mejora de desenlaces visuales.

Abstract :optic neuritis is uncommon as an ocular syphilis clinical presentation; the lack of typical features generates delay in the diagnosis. We describe the case of a 47-year-old woman, immunocompetent with a 4-month history of left visual acuity progressive of left side reduction, associated with pain, conjunctival injection and headache, physical examination with lumpy vision and fundus with signs of ocular inflammation, within the diagnostic evaluation, serum VDRL, FTA-ABS was reactive, with ANAS and IgG anticardiolipin antibodies serum positivity, lumbar punction was taken with reactive VDRL, optic neuritis by neurosyphilis was diagnosed, with antiphospholipid antibodies cross reactivity , treatment with crystalline penicillin 24 000 000 of units day for 14 days was started . In patients with signs of ocular inflammation, VDRL should be performed, confirmed with a treponemal test, and a lumbar puncture should be performed. Early treatment is associated with improvement of visual outcomes.

MOG-IgG associated optic neuritis is not multiple sclerosis / Neurite óptica associada ao MOG-IgG não é esclerose múltipla

ABSTRACT Autoantibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been reported in patients with inflammatory central nervous system disorders including isolated optic neuritis (ON). We compared our MOG-IgG ON patients with multiple sclerosis (MS) patients presenting with ON. Methods and results: Among the total of 38 patients with optic neuropathies, six patients with isolated ON were MOG-IgG positive and eight patients with ON fulfilled the diagnostic criteria for MS. All MS patients were negative for MOG-IgG using a cell-based assay. When compared with the MS group, the MOG-IgG patients were older (mean 47 years), more frequently male (ratio 2:1) and had a higher frequency of bilateral and/or recurrent ON. The brain magnetic resonance imaging of all MOG-IgG positive patients was normal or had only unspecific white matter T2 lesions. Conclusion: These findings suggest that MOG-IgG is a biomarker of an inflammatory demyelinating CNS disease distinct from MS.

RESUMO Autoanticorpos contra a glicoproteína da mielina do oligodendrócito (MOG-IgG) têm sido descritos em pacientes com neurite óptica (NO) isolada, entre outras doenças inflamatórias do sistema nervoso central. Comparamos os nossos pacientes com NO MOG-IgG positivos com pacientes com NO associada a esclerose múltipla (EM). Materias e métodos: De um total de 38 pacientes com neuropatia óptica, seis foram MOG-IgG positivos e oito preencheram critérios diagnósticos para EM. Todos os pacientes com EM foram negativos para MOG-IgG (ensaio baseado em células). Quando comparados ao grupo com EM, os pacientes MOG-IgG positivos apresentam idade mais avançada (mediana de 47 anos) e tiveram uma frequência maior de NO bilateral e/ou recorrente. Houve predomínio masculino (relação 2:1). A ressonância magnética de encéfalo de todos os pacientes MOG-IgG positivos foi normal ou demonstrou apenas lesões inespecíficas em T2. Conclusão: Nossos achados sugerem que o MOG-IgG é um biomarcador de doença desmielinizante diferente da EM.

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.

Perfil epidemiológico y clínico de los niños con la neuritis óptica del Hospital Nacional de Niños Benjamín Bloom de enero del 2009 a diciembre de 2013 / Epidemiological and clinical profile of children with optic neuritis from the Benjamín Bloom National Children's Hospital from January 2009 to December 2013

Se identificaron las manifestaciones clínicas y hallazgos en los exámenes de gabinete de la neuritis óptica. Asimismo se describió el perfil epidemiológico y clínico de los niños con neuritis óptica que podrá servir de base en estudios futuros y así realizar guías clínicas que ayuden a mejorar la calidad de vida de los niños que padezcan de la enfermedad y disminuir el retraso en la intervención diagnóstica e iniciar el tratamiento adecuado. Para esto se realizó un estudio descriptivo, tipo transversal retrospectivo, la población de estudio fueron los pacientes que consultaron en el Hospital Nacional de Niños Benjamín Bloom, con diagnóstico de neuritis óptica en los años 2009 al 2013. El método y recolección de datos se realizó a través de expedientes clínicos de pacientes que cumplieron con los criterios de inclusión. Se obtuvo 21 casos de neuritis óptica, el 67% de sexo femenino, 62% de los niños tenían entre 6 a 10 años. El síntoma predominante fue la afección bilateral, seguido de cefalea y disminución de la agudeza visual. El 86% presentaron potenciales evocados con resultados anormales; el tratamiento de elección fue el esteroide (prednisona/ metilprednisolona). Hubo mayor número de casos en el 2009, la punción lumbar no se efectuó en 19 casos y solo 1 fue positiva a bandas oliclonales. En los estudios de neuroimagen, ninguno presentó lesiones diseminadas en sistema nervioso central. La evaluación clínica a largo plazo no fue posible, por la inasistencia de los pacientes a los controles subsecuentes

Neurolupus and pregnancy: An ethical and therapeutic dilemma in a second level hospital / Neurolupus y embarazo: un dilema ético y terapéutico en un hospital de segundo nivel

Optic neuritis secondary to systemic lupus erythematosus is a rare manifestation with a prevalence of 1%. The case described concerns a patient who presented with optic neuritis associated with SLE. She was 19 weeks pregnant, and required pulses with methyl-prednisolone and cyclophosphamide, which are within the category D drugs used during pregnancy. Three weeks later, she presented with uterine activity, and went into labor, with a fetus of 22 weeks gestation and weighing 430 g being obtained, which died 48 h later. In medical practice there are ethical guidelines and economic obstacles to carrying out diagnostic and therapeutic protocols established by limiting clinical practice

La neuritis óptica secundaria a lupus eritematoso sistémico es una rara manifestación con una prevalencia del 1%. Presentamos el caso de una paciente que mostró neuritis óptica asociada a lupus eritematoso sistémico, con 19 semanas de gestación, requiriendo de pulsos de metilprednisolona y ciclofosfamida, que se encuentran dentro de los medicamentos categoría D utilizados durante el embarazo. Tres semanas después presentó actividad uterina y posteriormente trabajo de parto obteniéndose un producto de 22 semanas de gestación y 430 g, falleciendo a las 48 h. En la práctica médica existen lineamientos éticos y obstáculos económicos que limitan la realización de protocolos diagnósticos y terapéuticos establecidos, limitando la práctica clínica

Retinitis y fiebre por chikunguña: reporte de caso / Chikunguya fever and retinitis. Case report

Contexto: La enfermedad por Chikunguña es una infección viral, que ha sido un problema importante de salud en el mundo, recientemente asociado con manifestaciones oculares. Actualmente, no existe ningún tratamiento o vacuna para la fiebre por Chikunguña. Esta revisión se concentra en las manifestaciones de la retina de esta enfermedad viral a través de un caso clínico. La importancia de publicar este reporte es facilitar un diagnóstico precoz, basados en la poca evidencia que existe y así no sea una enfermedad subdiagnosticada. Presentación del caso: Paciente de sexo masculino de 46 años de edad, con antecedentes de trastorno bipolar tipo 2 quien experimenta fiebre, artralgias, rash cutáneo severo y edema en miembros inferiores, durante el brote de fiebre por Chikunguña, asociado con manifestaciones visuales: disminución de la agudeza visual, visión borrosa y escotoma en su ojo izquierdo. La Tomografía de coherencia óptica y la Angiografía fluoresceínica evidencian retinitis, y desprendimiento de vítreo posterior. Conclusiones: Los cambios oculares asociados con Chikunguña deben ser considerados en las regiones epidémicas. Existe la necesidad de dar importancia clínica, no sólo por el compromiso de esta enfermedad viral sistémica, sino también la presentación de manifestaciones oculares, y disminuir los casos de morbilidad y mortalidad

Background: Chikungunya is a viral infection that has been a major health world problem, recently associated with ocular manifestations. Currently, there is no treatment or vaccine for Chikungunya fever. In this work, we focus on the retinal manifestations of this viral disease, reporting a single clinical case. The importance of publish this report is to facilitate on early diagnosis, based on the lack of evidence and misdiagnosed. Case presentation: A 46 years old male, with history of bipolar II disorder, experienced fever, joint pain, severe rash and lower limb edema, during the outbreak of Chikungunya fever, associated with visual manifestations: decrease visual acuity, blurred vision and scotoma on his left eye; coherence tomography and fl uorescein angiography supports retinitis, and posterior vitreous detachment. Conclusions: Chikungunya associated ocular changes should be considered in epidemic regions. It is necessary to considered not only systemic manifestations but ocular, to reduce morbidity and mortality cases.

Mielopatía por enfermedad de devic: presentación de un caso y revisión de la literatura / Myelopathy Devic disease: case report and review of literature

La neuromielitis óptica, también conocida como enfermedad de Devic, es una enfermedad desmielinizante cuya prevalencia es muy baja. Se caracteriza por la inflamación y creación de anticuerpos contra el sistema nervioso central, respetando el cerebro pero comprometiendo el nervio óptico y dando alteraciones medulares. La presencia de anticuerpos de suero: acuaporina-4 de inmunoglobulina G es la razón por la que se diferenció de la esclerosis múltiple, del cual antes formaba parte como una variante de presentación clínica. El cuadro clínico de esta enfermedad se caracteriza por perdida de agudeza visual y mielitis transversa. Caso Clínico: Se presenta el caso de una paciente investigada por un periodo de aproximadamente 5 meses de duración sin factores de riesgo, acude a la Caja Petrolera de Salud por presentar dolor en región lumbar izquierda, de tipo opresivo, de alta intensidad y dificultad para pararse acompañada de paresia aguda de extremidad inferior izquierda, sin mejoría aún después de la administración de Quetorol®. Después de descartar varias enfermedades se llegó al diagnóstico de un posible diagnóstico de mielopatía asociada con la enfermedad de Devic. Discusión: Esta enfermedad es muy heterogénea en su presentación, por lo que los criterios diagnósticos se modificaron y se llegó a un nuevo consenso: La nueva nomenclatura define el término unificador Neuromielitis Óptica y el de Trastornos del espectro de Neuromielitis Óptica, que se estratifica aún más por las pruebas serológicas correspondientes a esta paciente apoyadas también en Resonancia Magnética; identificando este caso por métodos de exclusión.

Neuromyelitis Optica, also known as Devic's disease, is a demyelinating disease whose prevalence is very low; it is characterized by inflammation and the creation of antibodies against the central nervous system, respecting the brain but compromising the optic nerve and spinal cord changes giving.The presence of serum antibodies: aquaporin-4 immunoglobulin G is the reason why it differed from multiple sclerosis which was formerly part as a variant of clinical presentation.The clinical picture of this disease is characterized by loss of visual acuity and transverse myelitis. Clinical case: the case of a patient investigated for a period of approximately 5 months with no risk factors, goes to Caja Petrolera de Sauld because of pain in the left lumbar region, oppressive type, high intensity and difficulty is presented stand accompanied by acute left lower extremity paresis, with no improvement even after administration of Quetorol®, after discarding several diseases led to the diagnosis of a possible diagnosis of myelopathy associated with Devic's disease. Discussion:This disease is very heterogeneous in its presentation, so the diagnostic criteria were modified and reached a new consensus: The new nomenclature defines the unifying term Neuromyelitis Optica and Neuromyelitis Optica Spectrum Disorder, which stratifies more by serological tests for this patient also supported by Magnetic Resonance; identifying this case exclusion methods.