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.

Takayasu arteritis associated with ulcerative colitis and optic neuritis: first case in Korea

Takayasu arteritis (TA) is a chronic vasculitis that affects the aortic arch and its primary branches. Ulcerative colitis (UC) is an inflammatory bowel disease of unknown etiology. Patients diagnosed with both TA and UC have rarely been reported. The pathogenesis of TA and UC is uncertain, but cell-mediated mechanisms play an important role in both diseases, and a genetic factor is thought to have an effect on the coincidence of these two diseases. We herein report a 38-year-old female with TA who had a history of UC with optic neuritis. We believe that this is the first case of the coexistence of TA and UC in Korea.

Analysis of the Causes of Optic Disc Swelling

PURPOSE: To investigate the clinical manifestations and diagnoses of optic disc swelling. METHODS: The medical records of 49 patients who experienced optic disc swelling between March 2008 and June 2009 were retrospectively reviewed. The characteristics of non-arteritic anterior ischemic optic neuropathy (NA-AION) and optic neuritis (ON), which showed optic disc swelling most commonly, were compared. RESULTS: NA-AION was the most common disorder (34.7%) that presented with optic disc swelling. ON was identified in 15 patients (30.6%). Seven out of 49 patients (14.3%) had intracranially associated diseases, such as papilledema and compressive optic neuropathy. Pseudopapilledema was noted in four patients (8.2%). Other diseases (e.g., papillophlebitis, neuroretinitis, and diabetic papillopathy) were seen in six patients (12.2%). Ocular pain was observed more commonly in patients with ON (p = 0.001). Patients with ON expected a better visual prognosis than patients with NA-AION (0.12 +/- 0.32 vs. 0.49 +/- 0.35, p = 0.001). CONCLUSIONS: NA-AION and ON should be considered in the differential diagnosis when patients with optic disc swelling present to the neuro-ophthalmology clinic. Detailed history taking and supportive examinations, such as visual field, color-vision and imaging tests, should also be performed as indicated. Regular follow-up of such exams is necessary for the differential diagnosis of these diseases.

Neuritis óptica en niños: experiencia clínica en 13 años / Optic neuritis in children: clinic experience in 13 years

Background: In pediatric optic neuritis usually occurs after an infectious event, with papilledema, bilateral and with good prognosis, with a low incidence of conversion to multiple sclerosis. The aim of this paper is to present the clinical and laboratory characteristics of cases of optic neuritis in children. Patients and Methods: 10 clinical cases of optic neuritis in children and youth aged 5 to 17 years, referred between 1995 to 2007. Results: The median age at presentation was 11 years. 8 cases were females. Four cases with a history of respiratory infection, bilateral involvement in 8 of 10 patients and 5 cases of retrobulbar optic neuritis. Magnetic resonance imaging showed T2 hyperintensity in the optic nerves affected in 5 patients. The study of cerebrospinal fluid was normal and oligoclonal bands in all cases. The cases treated with intravenous methylprednisolone had good recovery. Two cases have multiple sclerosis. Discussion: In this series of female patients predominated and limited history of previous infection. Cases with multiple sclerosis showed no clinical differences, but more hyperintense lesions on magnetic resonance. The cases treated with methylprednisolone had good visual prognosis.

Antecedentes: En pediatría la neuritis óptica generalmente se presenta después de un cuadro infeccioso, con edema de papila, que suele ser bilateral y tiene buen pronóstico. La conversión a esclerosis múltiple es baja. Nuestro objetivo es presentar las características clínicas y de laboratorio de casos de neuritis óptica en pediatría. Pacientes y Método: Se analizan 10 casos clínicos de neuritis óptica en niños y jóvenes entre 5 y 17 años, referidos entre los años 1995 a 2008. Resultados: La edad media de la serie fue de 11 años. 8 casos eran de sexo femenino; cuatro tenían antecedentes de infección respiratoria. En 8 de 10 pacientes el compromiso fue bilateral y 5 casos evolucionaron con neuritis óptica retrobulbar. La resonancia magnética mostró hiperintensidad en T2 en los nervios ópticos afectados en 5 pacientes. El estudio de líquido cefalorraquídeo y bandas oligoclonales fue normal en todos los casos. Los pacientes tratados con metilprednisolona endovenosa tuvieron buena recuperación. Sólo dos casos evolucionaron a esclerosis múltiple. Discusión: En esta serie predonimaron los pacientes de sexo femenino y el antecedente infeccioso fue poco frecuente. Los casos que evolucionaron a esclerosis múltiple no mostraron diferencias clínicas; sólo presentaron mayor cantidad de lesiones hiperintensas en la RM. Tuvieron mejor pronóstico visual los enfermos tratados con metilprednisolona.

Vitreitis and movement disorder associated with neurosyphilis and human immunodeficiency virus (HIV) infection: case report / Vitreíte e distúrbio motor associados à neurosífilis e infecção pelo vírus da imunodeficiência humana (HIV): relato de caso

In this report, we describe an unusual patient with a choreiform movement disorder, misdiagnosed as Huntington disease, who later developed dense vitreitis leading to the identification of Treponema pallidum as the underlying pathogen of both abnormalities.

Neste relato descrevemos um caso infreqüente de um paciente com quadro de distúrbio motor coreiforme diagnosticado equivocadamente como doença de Huntington, o qual posteriormente desenvolveu quadro de intensa vitreíte, possibilitando a identificação do Treponema pallidum como o patógeno causador de ambas anormalidades.

Pediatric optic neuritis.

OBJECTIVE: Describe the clinical characteristics of pediatric optic neuritis. MATERIAL AND METHOD: Retrospective observational case series was performed on patients < or = 12 years of age with optic neuritis at Childrens Hospital Los Angeles. RESULTS: Thirty-one patients (48 eyes) were identified. Mean follow-up was 2.7 years. There were 17 preadolescents (< 10-years-old) in group I, and 14 adolescents (10--12-years-old) in group II. Females comprised 59% of group I, and 71% of group II. Bilateral cases comprised 65% from group I, and 43% from group II. Five patients from group I had acute disseminated encephalomyelitis (ADEM). Two patients from group II had multiple sclerosis (MS). No other patients developed MS. There was no difference in initial or final vision for the eyes with or without steroid treatment. CONCLUSION: Pediatric optic neuritis has no gender or racial predilection, is usually bilateral, and is associated with ADEM rather than MS.

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.

A Case of Optic Neuritis Associated with Crohn's Disease / 대한소화기학회지

In Crohn's disease, neurologic complications such as cerebrovascular accident, headache, peripheral neuropathy have been reported sporadically. The pathogenesis of these neurologic complications is still unknown and controversial. We experienced a 22-year-old man, with Crohn's disease accompanied by optic neuritis. Loss of visual acuity was developed during the worsening course of enterocutaneous fistula. After high dose steroid treatment, his visual acuity and neurologic symptoms improved immediately.

Neuritis óptica desmielinizante primaria: análisis de una experiencia con 209 pacientes atendidos en la unidad de neuro-oftalmología del Hospital Vargas de Caracas (1980-2001) / Neuritis optic primary dimyelinating: analysis of an experience with 209 patients assisted in the unit of neurophthalmoly of the Hospital Vargas Caracas (1980-2001)

La neuritis óptica desmielinizante primaria, es un proceso inflamatorio focal del nervio óptico aislado o parte de un proceso desmielinizante diseminado del sistema nervioso central: esclerosis múltiple. Un análisis retrospectivo de 209 pacientes atendidos en la Unidad de Neuro-oftalmología del Hospital Vargas de Caracas y centro privado, 1980 a 2001 describe características clínicas, evolución y relaciones. Observándose mayor incidencia en mujeres. Edad promedio 35.9 años. Formas intraocular, retrobulbar y atrofia óptica desmielinizante primaria muestran frecuencia similar. 119 (56,9 por ciento) compromiso unilateral y 84,5 por ciento constituyen primer episodio 90 pacientes (42,5 por ciento) compromiso bilateral, forma simultánea en 47 (52,2 por ciento). 49 por ciento tenían EM definida al momento del compromiso visual. Déficit visual central en 99,5 por ciento de los casos. Agudeza visual: mejor de 20/40 (33,66 por ciento) y cuenta-dedos o peor (39.3 por ciento). El 70 por ciento presenta defecto pupilar aferente relativo y discromatopsia. Escotoma central o centrocecal fue el hallazgo más frecuente. El 91,5 por ciento con agudeza visual inicia igual o mejor de 20/40, mejorando o no se modificaron. A los 6 meses de seguimiento, el 45,4 por ciento con agudeza de 20/200 o peor, mantuvieron el nivel visual inicial. A los 6 meses, 8 pacientes con visión inicial de "no-percepción-luminosa", no alcanzaron visión mejor de "cuenta-dedos". Difieren de series internacionales: porcentaje similar a formas intraoculares y retrobulbares, numero similar de afectación unilateral y bilateral inicial, especialmente mayor compromiso inicial de la agudeza visual y una pobre recuperación en pacientes con déficit importante de la agudeza visual. Las formas de extrema severidad y pronóstico visual sombrío, son frecuentes en nuestro medio

The clinical profile of childhood optic neuritis

PURPOSE: To report the clinical features and outcome of a series of children with optic neuritis. METHODS: We reviewed the medical records of patients up to 16 years old with optic neuritis. Group 1 comprised children seen up to two weeks after the onset of visual loss; Group 2 comprised patients already harboring optic atrophy. RESULTS: There were 15 boys and 12 girls. The mean age was 10.9 years. Bilateral optic neuritis occurred in 10. Optic disc pallor was found in 35 percent, edema in 46 percent, and 19 percent had normal fundus. During follow-up visual acuity improved in all but one eye in Group 1, and in six of seven eyes in children in Group 2. Just one child converted to multiple sclerosis. CONCLUSIONS: This study shows that the clinical features of childhood optic neuritis differ from those observed in adults. In children it has a better visual outcome and a lower conversion rate to multiple sclerosis than in adults

Síndrome de Miller Fisher e neurite óptica: relato de caso / Miller Fisher syndrome and optic neuritis: case report

Descreve-se um caso de síndrome de Miller Fisher associada a neuropatia óptica desmielinizante bilateral, confirmada pelo exame de potencial evocado visual, sugerindo possível comprometimento do sistema nervoso central nessa síndrome

Uso de los glucocorticoides en el tratamiento de la neuritis óptica / Use of glucocorticoids in optic neuritis treatment

Se presenta una síntesis del estudio multicéntrico de tratamiento de la neuritis óptica efectuado en varios centros de los Estados Unidos de Norteamérica. Se presentan las características clínicas de la neuritis óptica de este grupo de pacientes. Además se informa de los hallazgos neurológicos en el examen inicial, la incidencia de esclerosis múltiple, los hallazgos de la resonancia magnética cerebral y finalmente el valor terapéutico de los corticoides

The risk of multiple sclerosis developing in patients with isolated idiopathic optic neuritis in Brazil

O risco de progressäo da neurite óptica isolada (NOII) para esclerose múltipla (EM) ainda näo está bem definido, com taxas variáveis dependendo de critérios de diagnóstico, tempo de follow-up, fatores genéticos, raciais e possivelmente geográficos. Com o propósito de estudar a taxa de desenvolvimento de EM em pacientes com NOII, um grupo de 88 pacientes (52 homens e 36 mulheres) com NOII foi seguido por período de 1 mês a 9 anos (média 4,6 anos). Em 19 pacientes a neurite óptica foi bilateral, ou seja, ocorreu simultaneamente ou em intervalo até 4 semanas nos dois olhos; enquanto em outros 19 pacientes ela foi sequencial, afetando o olho contralateral após intervalo maior que um mês. Recorrências afetando o mesmo olho foram observadas em 7 pacientes. Nove pacientes (10,8%) com NOII desenvolveram sinais de EM entre 1 mês e 5 anos após o primeiro episódio de neurite óptica, o intervalo mediano sendo 1 ano. Estes pacientes representavam 13,9% das mulheres e 7,7% dos homens com NOII e a mediana das idades foi 25 anos. Dois terços dos pacientes que desenvolveram EM apresentavam envolvimento predominantemente medular. Nossos achados...

Sífilis ocular em pacientes infectados pelo HIV: relato de 2 casos e revisäo de literatura / Ocular syphilis in patients infected by HIV: a report of 2 cases and literature revision

Existe uma correlaçäo epidemiológica importante entre AIDS e sífilis. A infecçäo pelo vírus da imunodeficiência humana (HIV) acelera o curso natural da sífilis, fazendo com que estágios mais avançados da doença sejam atingidos mais precocemente. Os autores relatam 2 novos casos da associaçäo sífilis ocular/AIDS e fazem uma revisäo dos 22 casos anteriormente publicados. Discutem a possibilidade de alteraçäo das respostas imunológicas e enfatizam a alta incidência de neurossífilis, sugerindo que sempre se faça a análise do líquor quando se constatar essa associaçäo

Atrofia óptica pós-neurite induzida pelo etambutol, isoniazida e drogas correlatas / Optic atrophy induced for ethambutol, isoniazid and others drugs

O autor apresenta um caso de uso de drogas anti-microbianas usada na quimioterapia da tuberculose que levou a uma trofia óptica pós-neurite em ambos os olhos em uma paciente de 58 anos, do sexo feminino, branca, natural de Alegrete, RS