Enfermedad asociada a anticuerpos contra la glicoproteína de mielina de oligodendrocitos / Myelin oligodendrocyte glycoprotein antibody disease

Introducción: La enfermedad asociada a anticuerpos contra la glicoproteína de mielina del oligodendrocito (MOGAD, por sus siglas en inglés) es una entidad clínica recientemente identificada. La frecuencia de presentación del MOGAD es desconocida, pero se considera baja con respecto a otras enfermedades inflamatorias desmielinizantes. Materiales y métodos: Revisión narrativa de la literatura. Resultados: Las manifestaciones clínicas de esta condición son heterogéneas e incluyen neuritis óptica, mielitis, desmielinización multifocal del sistema nervioso central y encefalitis cortical. Se han descrito algunos hallazgos radiológicos que aumentan la sospecha diagnóstica, como el realce perineural del nervio óptico, el signo de la H en el cordón espinal y la resolución de lesiones T2 con el tiempo. El diagnóstico se basa en la detección de inmunoglobulinas G específicas contra MOG, en el contexto clínico adecuado. El tratamiento consiste en manejo de los ataques agudos con dosis altas de corticoides y en algunos casos se deberá considerar la inmunosupresión crónica, considerar la inmunosupresión crónica en pacientes con recurrencia o con discapacidad severa residual tras el primer evento. Conclusiones: En esta revisión narrativa se resumen los aspectos clave con respecto a la fisiopatología, las manifestaciones, el diagnóstico y el tratamiento de la MOGAD.

Introduction: The disease associated with antibodies against the myelin oligodendrocyte glycoprotein (MOGAD) is a recently identified clinical entity, with unknown frequency, but is considered low compared to other demyelinating inflammatory diseases. Materials And Methods: Narrative review. Results: The clinical manifestations are heterogeneous, ranging from optic neuritis or myelitis to multi-focal CNS demyelination or cortical encephalitis. There have been described characteristic MRI features that increase the diagnostic suspicion, such as perineural optic nerve enhancement, spinal cord H-sign or T2-lesion resolution over time. The diagnosis is based on the detection of specific G- immunoglobulins against MOG, in the suggestive clinical context. Acute treatment is based on high dose steroids and maintenance treatment is generally reserved for relapsing cases or patients with severe residual disability after the first attack. Conclusions: In this narrative review, fundamental aspects of pathophysiology, clinical and radiological manifestations, diagnosis and treatment of MOGAD are discussed.

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.

Recurrence factors for myelin oligodendrocyte glycoprotein antibody disease in children and the effect of recurrence prevention regimens / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features and recurrence factors of myelin oligodendrocyte glycoprotein (MOG) antibody disease in children and the effect of recurrence prevention regimens.@*METHODS@#A retrospective analysis was performed on the medical data of 41 children with MOG antibody disease who were hospitalized in the Department of Pediatric Neurology, Xiangya Hospital of Central South University, from December 2014 to September 2020. According to the presence or absence of recurrence, they were divided into a monophasic course group (@*RESULTS@#For these 41 children, acute disseminated encephalomyelitis was the most common initial manifestation and was observed in 23 children (56%). Of the 41 children, 22 (54%) experienced recurrence, with 57 recurrence events in total, among which optic neuritis was the most common event (17/57, 30%). The proportion of children in the recurrence group who were treated with corticosteroids for less than 3 months in the acute phase was higher than that in the monophasic course group (64% @*CONCLUSIONS@#More than half of the children with MOG antibody disease may experience recurrence. Most children with recurrence are treated with corticosteroids for less than 3 months in the acute phase. Rituximab and azathioprine may reduce the risk of recurrence.

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)

A Report of Two Case of Ocular Toxicity Resulting from Direct or Indirect Bee Venom

PURPOSE: To report a patient stung by a bee, who was diagnosed with sterile endopthalmitis and another patient diagnosed with optic neuritis, with decreasing visual acuity, after refined bee venom injection around the orbital tissue. CASE SUMMARY: A 82-year-old female visited our hospital for decreased visual acuity in the right eye and ocular pain due to a bee sting. The bee sting penetrated the sclera into the vitreous. In the anterior segment, severe cornea edema and anterior chamber cells were seen. Using ultrasonography, inflammation was seen around the intravitreal area. After 3 months, intravitreal inflammation regressed but the patient's visual acuity was light perception negative, and corneal opacity, neovascularization, and phthisis bulbi were detected. A 55-year-old male visited our hospital for ocular pain in the right eye and decreasing visual acuity after refined bee venom injection around the orbital tissue. The best-corrected visual acuity in the right eye was 15/100, there was moderate injection on the conjunctiva. A relative afferent pupillary defect, abnormal color vision test results, and a defect in the visual field test were observed. There was no pain during external ocular movement, and other general blood tests, and a brain MRI were normal. Based on these symptoms, methylprednisolone megatherapy was started for treatment of optic neuritis. After treatment, visual acuity of the right eye was 9/10 and all other clinical optic neuritis symptoms regressed. CONCLUSIONS: Based on these two cases, ocular toxicity from bee venom could result from both direct and indirect courses. Treatment using refined bee venom might be harmful, and caution is recommended in its use.

A Case of Leber Hereditary Optic Neuropathy Showing Optic Disc Hyperfluorescence

PURPOSE: We report an unusual case of Leber hereditary optic neuropathy presenting with optic disc hyperfluorescence. CASE SUMMARY: A 17-year-old male with sequential painless visual loss 3 weeks apart affecting first the left and then the right eye presented to our neuro-ophthalmology clinic. His best-corrected visual acuity was counting fingers in the right eye and 0.32 in the left eye. Fundus examination showed mild optic disc edema and hyperemia in both eyes, which were worse in the right eye. Fluorescein angiography revealed dye leakage from the right optic disc in the late phase. The results of magnetic resonance imaging of the brain and spinal cord were normal, and lumbar puncture study was unremarkable. Mitochondrial DNA sequencing revealed a pathognomonic 11778 mutation for Leber hereditary optic neuropathy. His vision deteriorated to 0.03 in both eyes 6 months later, but slowly started to improve 11 months after onset. At 2 years, his corrected visual acuity was 0.2 in both eyes. CONCLUSIONS: To our knowledge, this is the first report of optic disc hyperfluorescence in Leber hereditary optic neuropathy. This finding suggests that this mitochondrial optic neuropathy can masquerade as optic neuritis.

Optic Neuritis Accompanied by Optic Perineuritis after Peribulbar Retinalamin Injection

PURPOSE: To report a case of optic neuritis accompanied by optic perineuritis after retinalamin peribulbar injection. CASE SUMMARY: A 46-year-old Kazakhstan woman presented with sudden vision loss, headache, and pain in both eyes which began 2 hours after the peribulbar injection of retinalamin approximately 10 days prior to her initial visit. At the initial visit, the best-corrected visual acuity was light perception in both eyes. A fundus examination showed bilateral optic disc swelling and fluorescein angiography showed late-phase leakage around the optic nerve and optic nerve sheath enhancement. On brain magnetic resonance imaging, the tram track sign and donut sign were seen in both eyes. Steroid pulse therapy was started after the diagnosis of optic neuritis accompanied by optic perineuritis. After 6 months, the patient's visual acuity improved up to 0.8 in the right eye and 1.0 in the left eye. The light reflex was recovered, the relative afferent pupillary defect decreased, and there was no evidence of optic disc swelling. CONCLUSIONS: When an unverified drug injection is performed (e.g., retinalamin), it could result in complications such as optic neuritis and optic perineuritis from an allergic reaction or peribulbar injection trauma. In such cases, high-dose steroid pulse therapy may be considered.

Two Cases of Hypersensitivity Reactions Caused by Human Serum Albumin During Therapeutic Plasma Exchange

Iso-oncotic human serum albumin (HSA) is the primary replacement fluid of choice during therapeutic plasma exchange (TPE). Hypersensitivity reactions to HSA are rare, but require proper evaluation and management. In this article, we report two cases of hypersensitivity reactions to 5% HSA during TPE and discuss strategies to address this problem. The first case was a 60-year-old female patient, who was scheduled for TPE for treatment of recurrent focal segmental glomerulosclerosis after ABO-incompatible kidney transplantation. She developed a pruritic rash on her entire body during the first two sessions of TPE using 5% HSA. The third session was conducted using 500 mL normal saline, 1,000 mL 10% pentastarch, and 750 mL 5% HSA, where she eventually developed a pruritic rash when HSA was infused. There were no adverse events during the fourth and fifth session when fresh frozen plasma was used in place of HSA. The second case was a 50-year-old male patient diagnosed with optic neuritis, who was admitted for five sessions of TPE. The patient developed a pruritic rash on his entire body during the first session of TPE using 5% HSA. The patient experienced no adverse events during the following four sessions using fresh frozen plasma. Certain elements contained in HSA, such as albumin aggregates, prekallikrein activator, and caprylate-modified albumin, might be the reason for these hypersensitivity reactions. Careful selection of alternative replacement fluids is important to avoid premature termination of TPE procedures and secure optimal treatment options for patients.

Long-term follow-up of optic neuritis associated with meningoencephalitis of unknown etiology in a Maltese dog / 대한수의학회지

A 6-year-old intact male Maltese dog presented with a history of blindness and ataxia. Neuro-ophthalmic examination revealed dilated pupils with absent pupillary light reflexes and menace response in both eyes. Mild peripapillary edema was noted in the fundus of the right eye. After magnetic resonance imaging, the dog was provisionally diagnosed with meningoencephalitis of unknown etiology. Follow-up funduscopy was performed to monitor the condition of the optic discs for three years. Despite of the treatment with prednisolone, the optic nerve progressed to atrophy and the dog couldn't restore vision.

Association of Optic Neuritis with Neuromyelitis Optica Spectrum Disorder and Multiple Sclerosis in Korea

PURPOSE: To describe the clinical characteristics and course of optic neuritis (ON) and its association with neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) in Korea. METHODS: In this retrospective case series, 125 eyes of 91 Korean patients with ON were included. The medical documents of adult patients with ON were retrospectively reviewed. Patients were assigned into idiopathic ON, NMOSD, and MS groups according to the presence of an association with NMOSD or MS for subgroup analysis. Clinical characteristics, disease course, and visual and systemic prognosis were analyzed. RESULTS: During the mean follow-up of 3.7 years, 73 patients were diagnosed as idiopathic ON, 14 patients were diagnosed as NMOSD, and four patients developed definite MS. At the final visit, there were 13 (13%) eyes out of 100 eyes with idiopathic ON, nine (43%) eyes out of 21 eyes with NMOSD, and one (25%) eye out of four eyes with MS had a severe visual loss of 20 / 200 or less. The mean Expanded Disability Status Scale was 3.1 ± 1.5 in NMOSD and 1.8 ± 1.5 in the MS group at the final visit. In the NMOSD group, 50% of patients showed severe visual loss in at least one eye or were unable to ambulate without assistance at the final visit (5.3 ± 4.4 years after the initial episode of ON). CONCLUSIONS: Fourteen percent of patients showed positive results for NMO-immunoglobulin G test and 50% of patients with NMOSD showed a severe visual loss in at least one eye or were unable to ambulate without assistance. The proportion of MS was relatively low in Korean ON patients.

Screening of Workers with Presumed Occupational Methanol Poisoning: The Applicablility of a National Active Occupational Disease Surveillance System

BACKGROUND: Methyl alcohol poisoning in mobile phone–manufacturing factories during 2015–2016 was caused by methyl alcohol use for cleaning in computerized numerical control (CNC) processes. To determine whether there were health complications in other workers involved in similar processes, the Occupational Safety and Health Research Institute conducted a survey. METHODS: We established a national active surveillance system by collaborating with the Ministry of Employment and Labor and National Health Insurance Service. Employment and national health insurance data were used. Overall, 12,048 employees of major domestic mobile phone companies and CNC process dispatch workers were surveyed from 2016 to 2017. We investigated methyl alcohol poisoning by using the national health insurance data. Questionnaires were used to investigate diseases due to methyl alcohol poisoning. RESULTS: Overall, 24.9% of dispatched workers were employed in at least five companies, and 23.9% of dispatched workers had missing employment insurance history data. The prevalence of blindness including visual impairment, optic neuritis, visual disturbances, and alcohol toxicity in the study participants was higher than that reported in the national health insurance database (0.02%, 0.07%, 0.23%, and 0.03% versus 0.01%, 0.07%, 0.13%, and 0.01%, respectively, in 2015). Moreover, 430 suspicious workers were identified; 415 of these provided an address and phone number, of whom 48 responded (response rate, 11.6%). Among the 48 workers, 10 had diseases at the time of the survey, of whom 3 workers were believed to have diseases related to methyl alcohol exposure. CONCLUSION: This study revealed that active surveillance data can be used to assess health problems related to methyl alcohol poisoning in CNC processes and dispatch workers.

MRI Histogram Texture Feature Analysis of the Optic Nerve in the Patients with Optic Neuritis / 中国医学科学杂志(英文版)

Objective To evaluate the optic nerve impairment using MRI histogram texture analysis in the patients with optic neuritis.Methods The study included 60 patients with optic neuritis and 20 normal controls. The coronal T2 weighted imaging (T2WI) with fat saturation and enhanced T1 weighted imaging (T1WI) were performed to evaluate the optic nerve. MRI histogram texture features of the involved optic nerve were measured on the corresponding coronal T2WI images. The normal optic nerve (NON) was measured in the posterior 1/3 parts of the optic nerve. Kruskal-Wallis one-way ANOVA was used to compare the difference of texture features and receiver operating characteristic (ROC) curve were performed to evaluate the diagnostic value of texture features for the optic nerve impairment among the affected optic nerve with enhancement (ONwEN), affected optic nerve without enhancement (ONwoEN), contralateral normal appearing optic nerve (NAON) and NON.Results The histogram texture Energy and Entropy presented significant differences for ONwEN vs. ONwoEN (both P=0.000), ONwEN vs. NON (both P=0.000) and NAON vs. NON (both P<0.05). ROC analysis demonstrated that the area under the curve (AUC) of histogram texture Energy were 0.758, 0.795 and 0.701 for ONwEN vs. ONwoEN, ONwEN vs. NON and NAON vs. NON, AUC of Entropy were 0.758, 0.795 and 0.707 for ONwEN vs. ONwoEN, ONwEN vs. NON and NAON vs. NON.Conclusions The altered MRI histogram texture Energy and Entropy could be considered as a surrogate for MRI enhancement to evaluate the involved optic nerve and normal-appearing optic nerve in optic neuritis.