Encefalomielite aguda disseminada com provável etiologia de Bartonella henselae / Acute disseminated encephalomyelitis with probable Bartonella henselae etiology

RESUMO A encefalomielite aguda disseminada é uma doença rara, aguda, inflamatória e desmielinizante do sistema nervoso central, presumivelmente associada, em mais de três quartos dos casos, a infecções (virais, bacterianas ou inespecíficas) e imunizações ou sem qualquer antecedente indentificável. Habitualmente, apresenta um curso monofásico com início de sintomas inespecíficos na fase prodrómica, podendo evoluir com alterações neurológicas multifocais e até à perda total da acuidade visual. Descrevemos o caso de um menino de 9 anos com quadro inicial de edema de papila causado por encefalomielite aguda disseminada devido a Bartonella henselae. Apesar da gravidade da doença, o diagnóstico e o tratamento precoce proporcionaram bons desfechos.

ABSTRACT Acute disseminated encephalomyelitis is a rare, acute, inflammatory, and demyelinating disease of the central nervous system. Presumably associated in more than three quarters of cases by infections (viral, bacterial, or nonspecific) and immunizations or without any identifiable antecedent. It usually presents a monophasic course with onset of nonspecific symptoms in the prodromal phase and may evolve with multifocal neurological changes and even visual acuity loss. We describe a case of a 9-year-old boy with an initial picture of papillary edema caused by acute disseminated encephalomyelitis due to Bartonella henselae. Despite the severity of the disease, early diagnosis and treatment provided good outcomes.

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.

Clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease in Brazil: a single-center experience / Espectro clínico de doença associada com anticorpos contra a glicoproteína de oligodendrócito de mielina no Brasil: uma experiencia de centro único

Abstract Background Anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibody-associated disease (MOGAD) is an immune-mediated neurological disorder with a broad spectrum of clinical presentation that is often difficult to distinguish from other demyelinating diseases, such as multiple sclerosis and neuromyelitis optica spectrum disorder. Objective To describe the clinical and paraclinical characteristics of MOGAD in a Brazilian tertiary center. Methods We retrospectively reviewed the records of adult and pediatric patients who tested positive for anti-MOG antibodies and presented with clinical and radiological diseases compatible with MOGAD. Results Forty-one patients (10 children) were included: 56% female, 58% Caucasian, mean age at onset 31 years (range 6-64), with a mean disease duration of 59.6 months (range 1-264 months). The most frequent onset presentation was optic neuritis (68%), acute disseminated encephalomyelitis (ADEM, 12%), and myelitis (10%). A monophasic disease course was observed in 49%. EDSS median was 2.1 at the last visit. Most patients (83%) were under continuous immunosuppressive treatment. Azathioprine was the first-line treatment in 59%. In all ADEM cases, conus, and root involvement was radiologically observed on MRI. Conclusion Brazilian MOGAD patients presented with a similar spectrum of previously reported MOGAD phenotypes. Conus and spinal root involvement seems to be frequently present in MOGAD-ADEM and could serve as radiologic characteristics of this clinical entity.

Resumo Antecedentes A doença associada ao anticorpo da glicoproteína da mielina de oligodendrócitos (anti-MOG; MOGAD) é uma doença neurológica imunomediada com um amplo espectro de apresentações clínicas que muitas vezes é difícil de distinguir de outras doenças desmielinizantes, como a esclerose múltipla e o distúrbio do espectro da neuromielite óptica. Objetivo Descrever as características clínicas e paraclínicas da MOGAD em um centro terciário brasileiro. Métodos Revisamos retrospectivamente os prontuários dos pacientes adultos e pediátricos que testaram positivos para anticorpos anti-MOG e apresentaram um quadro clínico e radiológico compatível com MOGAD. Resultados Quarenta e um pacientes (10 crianças) foram incluídos: 56% do sexo feminino, 58% caucasianos, idade média de início da doença foi 31 anos (intervalo de 6-64), com duração média da doença de 59,6 meses (intervalo de 1-264 meses). A apresentação inicial mais frequente foi neurite óptica (68%), seguida pela encefalomielite disseminada aguda (ADEM, 12%) e mielite (10%). Um curso monofásico da doença foi observado em 49%. EDSS foi de 2,1 na última visita. A maioria dos pacientes (83%) estava sob tratamento imunossupressor contínuo. Azatioprina foi o tratamento de primeira linha em 59%. Em todos os casos de ADEM, o envolvimento do cone medular e das raízes espinhais foi observado radiologicamente na ressonância magnética. Conclusão Os pacientes brasileiros com MOGAD apresentam um espectro clínico e radiológico semelhante aos fenótipos de MOGAD relatados anteriormente. O envolvimento do cone e das raízes espinhais parece estar frequentemente presente no MOGAD-ADEM e poderia servir como característica radiológica nesta entidade.

Clinical features and visual outcomes of pediatric optic neuritis in the Indian population: A prospective study

Purpose: Optic neuritis, defined as inflammation of the optic nerve, is the most common optic neuropathy affecting adults. Various studies in Southeast Asia have shown that the clinical profile of optic neuritis might differ in these regions from that reported in the western literature. Through this study, we evaluate the clinical profile of pediatric optic neuritis (PON) in the Indian population. Methods: This was a hospital?based prospective observational study. Patients with optic neuritis younger than 16 years who attended the neuro?ophthalmology clinic from May 2016 to April 2017 were included in the study. Results: This study included 54 eyes of 38 patients. The mean age of presentation was 10.6 years. Unilateral disease (58%) was found to be more common, and a slight female preponderance (58%) was noted. The most common feature was visual loss (96.3%). Pupillary light reflex abnormality was seen in most patients. Fundus examination revealed disk edema (77.7%) to be the most common feature. Neuroimaging was performed in 34 patients, and multiple sclerosis was diagnosed in four patients. At 3 months follow?up after treatment, 89% of eyes had best correct visual acuity of 6/9 or better (P < 0.001). Conclusion: In our study, we found the clinical profile of PON to be similar to that seen in western studies as well as those done previously in the Indian population, although with a few differences

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.

Intoxicación por metanol por consumo de bebidas alcohólicas adulteradas. Efectos en el sistema nervioso. Reporte de un caso / Methanol Poisoning by Consumption of Adulterated Alcoholic Beverages. Effects on the Nervous System. A Case Report

Resumen La intoxicación por metanol puede ocurrir de forma inadvertida por la ingesta de bebidas alcohólicas adulteradas. Se trata una entidad poco frecuente, sin embargo, se ha reportado un aumento en la incidencia durante la pandemia de COVID-19. La intoxicación con metanol representa una urgencia médica, ya que puede provocar daño severo en el sistema nervioso central y periférico, además de acidosis metabólica, daño renal agudo e incluso la muerte. En este artículo se presenta el caso de un paciente que cursó con intoxicación por metanol de manera inadvertida al consumir bebidas alcohólicas presumiblemente adulteradas. En el encéfalo se demostró necrosis hemorrágica de ambos núcleos putamen, además de cursar con neuritis óptica bilateral y polineuropatía periférica. Fue manejado con pulsos de esteroides intravenosos, con lo cual, mejoró significativamente su función visual, sensitiva y motora. En el presente caso no existieron complicaciones fatales y presentó una buena respuesta al tratamiento, sin embargo, el caso pone de relieve la necesidad de una mejor regulación en la producción y comercialización de bebidas alcohólicas en nuestro país, y, por otro lado, permite hacer a un llamado a los consumidores a tomar más precauciones en el consumo de bebidas alcohólicas de dudosa calidad o procedencia.

Abstract Methanol poisoning can occur unnoticed, by the ingestion of adulterated alcoholic beverages. In general, it is a rare entity, however, an increase in incidence has been reported during the SARS-CoV-2 pandemic. Methanol poisoning represents a medical emergency as it can cause severe damage to the central and peripheral nervous systems, as well as metabolic acidosis, acute kidney injury, and even death. This article presents the case of a patient who inadvertently developed methanol intoxication after consuming presumably adulterated alcoholic beverages. In the brain, hemorrhagic necrosis of both putamen nuclei was demonstrated, in addition to presenting with bilateral optic neuritis and peripheral polyneuropathy. He was managed with intravenous steroid pulses, which significantly improved his visual, sensory, and motor function. In the present case there were no fatal complications and presented a good response to treatment, however, the case highlights the need for better regulation in the production and marketing of alcoholic beverages in our country, and on the other hand, to invite consumers to take more precautions in the consumption of alcoholic beverages of dubious quality or origin.

Neuromyelitis optica spectrum disorders: a review with a focus on children and adolescents / Espectro da neuromielite óptica: uma revisão com ênfase em crianças e adolescentes

Abstract Neuromyelitis optica spectrum disorder (NMOSD) is a rare and severe inflammatory disorder of the central nervous system (CNS). It is strongly associated with anti-aquaporin 4 antibodies (AQP4-IgG), and it mainly affects young women from non-white ethnicities. However, ~ 5 to 10% of all cases have onset during childhood. Children and adolescents share the same clinical, radiologic, and laboratory presentation as adults. Thus, the same NMOSD diagnostic criteria are also applied to pediatric-onset patients, but data on NMOSD in this population is still scarce. In seronegative pediatric patients, there is a high frequency of the antibody against myelin oligodendrocyte glycoprotein (MOG-IgG) indicating another disease group, but the clinical distinction between these two diseases may be challenging. Three drugs (eculizumab, satralizumab, and inebilizumab) have been recently approved for the treatment of adult patients with AQP4-IgG-positive NMOSD. Only satralizumab has recruited adolescents in one of the two pivotal clinical trials. Additional clinical trials in pediatric NMOSD are urgently required to evaluate the safety and efficacy of these drugs in this population.

Resumo O espectro da neuromielite óptica (ENMO) é uma rara e grave doença inflamatória do sistema nervoso central (SNC), fortemente associada ao anticorpo anti-aquaporina 4 (AQP4-IgG) e que afeta preferencialmente mulheres jovens de etnias não-caucasianas. No entanto, aproximadamente de 5 a 10% de todos os casos se iniciam na infância. Crianças e adolescentes compartilham as mesmas características clínicas, radiológicas e laboratoriais dos adultos. Além disso, o mesmo critério diagnóstico de ENMO é aplicado para pacientes com início na infância. No entanto, dados da população pediátrica são escassos. Em pacientes pediátricos soronegativos, existe uma alta frequência de positividade ao anticorpo contra a glicoproteína na mielina do oligodendrócito (MOG-IgG), indicando outra patologia; porém, a distinção clínica entre as duas doenças é desafiadora. Três medicações (eculizumabe, inebilizumabe e satralizumabe) foram recentemente aprovadas para pacientes adultos com AQP4-IgG. Apenas um dos ensaios pivotais do satralizumabe recrutou adolescentes. Novos ensaios clínicos em pacientes pediátricos com ENMO são necessários para avaliar a segurança e eficácia destas drogas nesta população.

Neuromielitis óptica y lupus eritematoso sistémico: caso clínico

La neuromielitis óptica (NMO) es una enfermedad desmielinizante del sistema Nervioso Central, con morbilidad, mortalidad alta, con respuesta favorable al tratamiento inmunosupresor y asociación infrecuente a enfermedades inmunológicas como Lupus Eritematoso Sistémico. Se reporta caso de un adolescente con diagnóstico de Neuromielitis óptica que presento durante su evolución Lupus Eritematoso Sistémico. Su evolución fue adecuada por la respuesta favorable a terapia inmunosupresora.

Optic neuromyelitis (NMO) is a demyelinating disease of the Central Nervous System, with morbidity, high mortality, favorable response to immunosuppressive treatment and infrequent association to immunological diseases such as Systemic Lupus Erythematosus. We report the case of a teenager with a diagnosis of Optic Neuromyelitis who presented Systemic Lupus Erythematosus during his evolution. Its evolution was adequate due to the favorable response to immunosuppressive therapy.

Dental erosion caused by glucocorticoid therapy in a patient with optic neuritis: a case report / 华西口腔医学杂志

Dental erosion is characterized by progressively destroyed teeth, which has no relation to bacteria but to chemicals. Some internal factors, such as gastroesophageal reflux induced by bulimia, anorexia, gastrointestinal diseases, or drugs, and external factors, such as diet, drugs, and occupational acid exposure, are considered promotive factors for this disease. This article presents a patient suffering from severe dental erosion in the whole dentition, especially in the maxillary teeth, due to gastroesophageal reflux induced by glucocorticoid therapy for optic neuritis. This article discusses the mechanism between optic neuritis glucocorticoid therapy and dental erosion.

Clinical and imaging features of infiltrative optic neuropathy secondary to extraocular malignant tumors / 中华眼底病杂志

Objective:To observe the clinical and imaging features of infiltrative optic neuropathy (ION) secondary to extraocular malignant tumors.Methods:A retrospective case study. From January 2017 to October 2022, 26 eyes of 20 patients with ION secondary to extraocular malignancies and 32 eyes of 16 patients with early papilloedema (EP) secondary to intracranial metastatic carcinoma were included in the study. All eyes underwent best corrected visual acuity (BCVA), fundus color photography, orbital and/or craniocerebral magnetic resonance imaging (MRI). A total of 54 eyes were examined by visual field examination, among which ION and EP were 22 and 32 eyes, respectively. Clinical and imaging features of the affected eye were retrospectively analyzed.Results:Among 26 eyes of 20 ION patients, there were 13 males and 7 females, with the mean age of (52.8±16.9) years. There were 10 patients of hematologic malignancy, 7 patients of periorbital malignancy, 2 patients of lung cancer, 1 patient of gastric cancer, 1 patient of breast cancer and 1 patient of prostate cancer. Two patients of nasal lymphoma were recorded as hematologic malignancies and periorbital malignancies. Sixteen patients had a history of systemic or periorbital malignancy, among which 4 patients reported that they had been "clinically cured". Optic neuritis was diagnosed in 15 patients. Among the 16 patients with EP, 5 were males and 11 were females, with the mean age of (47.9±12.3) years. The primary malignant tumors were lung cancer, breast cancer, leukemia, gastric cancer, ovarian cancer, colon cancer and rectal cancer in 7, 2, 2, 2, 1, 1, 1, respectively. In 26 eyes of ION, 20 eyes complained of blurred vision or peripheral vision occlusion and progressive aggravation; no obvious visual symptoms in 6 eyes. BCVA was light sensing to 1.0 with a median of 0.3, including light sensing and light sensing in 4 eyes. Optic disc edema was observed in 19 eyes; no obvious abnormality in 7 eyes. Visual field examination showed that in 22 eyes, normal or mild enlargement of blind spot in 3 eyes, arcuate scotoma in 4 eyes, annular scotoma in 6 eyes, tubular visual field or concentric contraction of visual field in 6 eyes, and diffuse depression in 3 eyes. MRI showed optic nerve enlargement with sheath enhancement in all ION eyes. Among 32 eyes of EP, 28 eyes showed recurrent transient amaurosis, and the other 4 eyes showed horizontal diplopia. BCVA ranged from 0.8 to 1.5, with a median of 1.0. All EP patients showed different degrees of optic disc hyperemia and edema by fundus examination. The visual field examination showed normal or mild enlargement of the physiological blind spot. MRI showed thickening of the optic nerve and widening of the intrathecal space, but no obvious enhancement of the optic nerve and its intrathecal membrane, and obviously enhanced space-occupying lesions in the brain parenchyma, accompanied by compression and edema of the surrounding brain tissue and midline displacement.Conclusions:ION secondary to extrocular malignant tumors mainly manifested as mild visual symptoms and obvious optic disc edema. MRI showed thickened optic nerve and strengthened sheath, and no obvious abnormality in optic nerve parenchyma.

Changes of corneal nerve fiber and the correlation analysis with visual acuity in demyelinating optical neuritis patients / 中华眼底病杂志

Objective:To investigate the changes in the nerve fiber layer of the cornea in patients with demyelinating optic neuritis (DON) and its correlation with visual acuity.Methods:A cross-sectional study. From March 2021 to July 2022, 27 cases (39 eyes) of DON patients diagnosed in the Department of Neurology and Ophthalmology of Beijing Tongren Hospital Affiliated to Capital Medical University were enrolled in this study. According to the serological test results, the patients were divided into aquaporin 4 antibody associated optic neuritis (AQP4-ON group) and myelin oligodendrocyte glycoprotein antibody associated optic neuritis (MOG-ON group), with 15 cases (19 eyes) and 12 cases (20 eyes) respectively. According to previous history of glucocorticoid treatment, the patients were divided into glucocorticoid treated group and non-glucocorticoid treated group, with 17 cases (27 eyes) and 10 cases (12 eyes) respectively. Twenty healthy volunteers (20 eyes) with age- and gender-matched were selected as the control group. All eyes underwent best corrected visual acuity (BCVA) and in vivo confocal microscopy (IVCM) examinations. BCVA was performed using Snellen's standard logarithmic visual acuity chart, which was converted into logarithmic minimum angle resolution (logMAR) visual acuity during statistics. The corneal nerve fiber length (CNFL), corneal nerve fiber density (CNFD), corneal nerve fiber branch length (CNBL), corneal nerve fiber branch density (CNBD) and the density of corneal dendritic cells (DC) were detected by IVCM examination. Parameter comparison between groups by t-test and Kruskal-Wallis rank sum test. The correlation between logMAR BCVA and pamameters of corneal nerve fibers were analyzed using Spearman analysis. Results:The CNFL, CNFD, and CNBL of the DON group and the control group were (10.67±2.55) mm/mm 2, (57.78±12.35) root/mm 2, (3.27±1.34) mm/mm 2, and (13.74±3.05) mm/mm 2, (70.95±13.14) root/mm 2, and (4.22±1.03) mm/mm 2, respectively; the difference in CNFL, CNFD, and CNBL between the two groups were statistically significant ( t=4.089, 3.795, 2.773; P<0.05). The CNFL, CNBL, and CNBD of the affected eyes in the MOG-ON group and AQP4-ON group were (12.02±2.13) mm/mm 2, (3.80±1.19) mm/mm 2, (47.97±8.86) fibers/mm 2, and (9.25±2.19) mm/mm 2, (2.72±1.19) mm/mm 2, (39.43±13.86) fibers/mm 2, respectively; the differences in CNFL, CNBL, and CNBD between the two groups were statistically significant ( t=-4.002, -2.706, -2.306; P<0.05). The corneal DC density of the patients in the hormone treated group and the non-hormone treated group was (24.43±8.32) and (41.22±9.86) cells/mm 2, respectively. The difference in corneal DC density between the two subgroups was statistically significant ( P<0.001). Correlation analysis showed that there was a significant negative correlation between logMAR BCVA and CNBL and CNFL in patients with DON ( r=-0.422, -0.456; P<0.05). Conclusions:There are different degrees of corneal nerve fiber damage in patients with different types of DON. There was a negative correlation between BCVA and the length of corneal nerve fibers.

Screening and diagnostic system construction for optic neuritis and non-arteritic anterior ischemic optic neuropathy based on color fundus images using deep learning / 中华眼底病杂志

Objective:To construct and evaluate a screening and diagnostic system based on color fundus images and artificial intelligence (AI)-assisted screening for optic neuritis (ON) and non-arteritic anterior ischemic optic neuropathy (NAION).Methods:A diagnostic test study. From 2016 to 2020, 178 cases 267 eyes of NAION patients (NAION group) and 204 cases 346 eyes of ON patients (ON group) were examined and diagnosed in Zhongshan Ophthalmic Center of Sun Yat-sen University; 513 healthy individuals of 1 160 eyes (the normal control group) with normal fundus by visual acuity, intraocular pressure and optical coherence tomography examination were collected from 2018 to 2020. All 2 909 color fundus images were as the data set of the screening and diagnosis system, including 730, 805, and 1 374 images for the NAION group, ON group, and normal control group, respectively. The correctly labeled color fundus images were used as input data, and the EfficientNet-B0 algorithm was selected for model training and validation. Finally, three systems for screening abnormal optic discs, ON, and NAION were constructed. The subject operating characteristic (ROC) curve, area under the ROC (AUC), accuracy, sensitivity, specificity, and heat map were used as indicators of diagnostic efficacy.Results:In the test data set, the AUC for diagnosing the presence of an abnormal optic disc, the presence of ON, and the presence of NAION were 0.967 [95% confidence interval ( CI) 0.947-0.980], 0.964 (95% CI 0.938-0.979), and 0.979 (95% CI 0.958-0.989), respectively. The activation area of the systems were mainly located in the optic disc area in the decision-making process. Conclusion:Abnormal optic disc, ON and NAION, and screening diagnostic systems based on color fundus images have shown accurate and efficient diagnostic performance.

Optic neuritis in Guillain-Barré syndrome: a case report and literature review / 中华神经科杂志

Objective:To summarize the clinical characteristics of patients with Guillain-Barré syndrome (GBS) complicated with optic neuritis (ON).Methods:The clinical data of a patient with GBS complicated with ON, who admitted to the Department of Neurology, the First Hospital of Shanxi Medical University in December 2021, were collected, including demographic characteristics, clinical symptoms and signs, laboratory and electrophysiological data, and results of fundus color films. The patients with GBS complicated with ON reported in the literature were also reviewed.Results:A 40-year-old female patient with GBS was diagnosed by the results of electromyography and cerebrospinal fluid tests combining with the history and signs, who was treated with intravenous immunoglobulin on the 3rd day after onset. On the 8th day, her muscle strength improved significantly. However, on the 12th day, the visual field darkened, and on the 19th day, the vision decreased significantly (oculus dexter visual acuity 0.2, oculus sinister visual acuity 0.1 +1) with bilateral papilloedema, a relative afferent pupillary defect and delayed P100 response of the visual evoked potential. Obvious abnormality was not noted in optic nerve magnetic resonance imaging. Thus ON was diagnosed and treated with pulse methylprednisolone therapy. After 8 days of treatment, the visual acuity was completely recovered and there was no abnormality in the ocular fundus. A total of 28 cases of GBS complicated with ON (including the present patient) were reported in the literature. The age of onset was mostly 20-60 years, and there was no gender preference. Mycoplasma pneumoniae was the most common premorbid pathogen and was identified in 7 of the 10 cases in which the causative agent was described. ON usually involved both sides, and 21 of 28 patients had bilateral optic nerves involved. GBS preceded ON or both occurred simultaneously in the majority of patients; GBS preceded ON in 14 of 28 patients, and both occurred simultaneously in 10 of 28 patients. All patients responded well to immunotherapy, and vision was completely recovered in 20 patients. Conclusions:GBS complicated with ON is rare. Attention should be paid to the loss of vision in patients with GBS. Relevant examinations should be completed as soon as possible and immunotherapy should be given.

Autoimmune optic neuropathy as the differential diagnosis of neuromyelitis optica spectrum disorders / Neuropatia óptica autoimune como diagnóstico diferencial do espectro da neuromielite óptica

ABSTRACT Optic neuritis is an important cause of decreased vision due to inflammation of the optic nerve. In view of its complex etiology, a thorough clinical evaluation is essential. Autoimmune optic neuropathy, a rare form of optic neuritis, is associated with progressive, painless, and severe visual loss. Severity depends on the inflammatory and ischemic components of the condition. Autoimmune optic neuropathy is ideally diagnosed with autoimmune disease markers (usually elevated levels of antinuclear antibodies). The treatment is immunosuppression with high doses of corticosteroids. Corticoid dependence is a characteristic of autoimmune optic neuropathy. In this report, we describe a patient with autoimmune optic neuropathy and discuss the importance of laboratory parameters and magnetic resonance imaging findings in the diagnosis of the disease.

RESUMO A Neurite óptica é uma importante causa de diminuição da visão devido à inflamação do nervo óptico. Por apresentar diversas etiologias faz-se necessário ampla investigação. A neuropatia óptica autoimune corresponde a uma doença rara que se manifesta com perda visual aguda, indolor e grave. A gravidade está associada a sua fisiopatogenia com componentes inflamatório e isquêmico. A positividade para marcadores de doenças autoimunes, mais comumente a elevação da titulação de anticorpos antinucleares, são fatores determinantes para o diagnóstico da neuropatia óptica autoimune. O tratamento é feito através de imunossupressão, com necessidade de altas doses de corticoide. Neste relato iremos descrever um paciente com neuropatia óptica autoimune. Discutiremos sobre a importância dos parâmetros laboratoriais e os achados de imagem da ressonância magnética para o diagnóstico.

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.

A case report of pediatric mog-antibody disease with Bilateral optic neuritis

MOG – Antibody disease is an inflammatory demyelinating condition of the CNS characterized by a monophasic or relapsing course of neurological dysfunction which does not meet the typical criteria for multiple sclerosis or other known neuro inflammatory conditions and occurs in presence of serum MOG antibodies using specific cell based assays. In pediatric patients MOG antibodies are detected in range of relapsing phenotypes including relapsing inflammatory optic neuritis (RION), acute disseminated encephalomyelitis followed by optic neuritis (ADEM – ON), brain stem demyelination and aquaporin P4 antibody negative neuromyelitis optica spectrum disorder (AQP4-Ab negative NMOSD).MOG positive optic neuritis is frequently bilateral and associated with optic nerve head swelling.It is associated with neurological diseases like Multiple Sclerosis, ADEM or Transverse Myelitis.MOG antibody IgG is detected in serum by indirect fluorescence test.IV Methylprednisolone is the treatment of choice, if it fails to improve vision or if optic neuritis is recurring, then a combination of plasma exchange and IV Methylprednisolone should be considered.Long term immunosuppressants used for Prevention include corticosteroids, azathioprine, mycophenolate mofetil and rituximab. The optimal preventive therapy has yet to be determined.Once the disease has been diagnosed, uncertainty remains over the best treatment approach and clinical trials for the pharmacological management of MOG- antibody optic neuritis are still needed

CNS Tuberculosis

Tuberculosis can involve almost any organ of the body. In the Central Nervous System (CNS) it can cause meningitis, tuberculoma, abscess,spondylitis, arachnoiditis, myeloradiculitis or other manifestations. Around 10% of all patients with tuberculosis have CNS involvement. Tuberculosis is rampant in the developing world and has reemerged as a major public health menace with the HIV pandemic. Compared with HIV-negative individuals, HIVpositive individuals with TB are 5 times more likely to have CNS involvement. Laboratory confirmation of CNS TB is difficult and hence empirical treatment has to be initiated as early as possible based on clinical and radiological features. In this article,we review the CNS manifestations of tuberculosis and their diagnosis and treatment

Neurosífilis con neuropatía óptica y auditiva simultáneas en un paciente VIH positivo / Neurosyphilis with simultaneous optic and auditive involvement in an HIV-positive patient

Introducción: a partir del año 2000, los médicos han asistido a un retorno de la sífilis vinculado a prácticas sexuales no protegidas y con parejas múltiples, en especial entre hombres que tienen sexo con hombres. La coinfección Treponema pallidum/virus de la inmunodeficiencia humana (VIH) modifica tanto la historia natural de la sífilis, incrementando la incidencia de neurosífilis temprana, como la respuesta al tratamiento con penicilina. Métodos: un paciente varón, peruano, de 36 años, seropositivo para VIH, consulta por dis-minución de la agudeza visual en ojo derecho, pérdida de la audición, tinnitus, mareos y vértigo. Refería antecedentes de sífilis en los 2 años previos. Resultados: el examen oftalmológico efectuado al paciente mostró células en el segmento anterior del ojo derecho. El fondo de ojo reveló la existencia de inflamación del nervio óptico asociada con panuveítis. En base a los hallazgos clínicos, los valores de VDRL en suero y líquido cefalorraquídeo (LCR) se diagnosticó neurosífilis (NS) con neuritis óptica, panuveítis y otosífilis en un paciente coinfectado por VIH. El paciente fue tratado con penicilina G sódica intravenosa, 4 millones de UI cada 4 horas, durante 2 semanas con buena respuesta clínica. Discusión: el compromiso de los pares craneales óptico y auditivo puede representar la manifestación de una NS temprana, en especial, en el contexto de un paciente VIH positivo. De acuerdo con nuestro conocimiento, este sería el segundo caso publicado de compromiso simultáneo del nervio óptico y del aparato vestíbulo-coclear en un paciente con NS.

Introduction: from the 2000s, the physicians experienced a return of syphilis, which may be related to unrestricted sexual behaviour with unprotected contact between multiple partners, especially in men who have sex with men. Concurrent infection with human immunodeficiency virus (HIV) alters the natural history of syphilis by increasing the frequency of early neurosyphilis and the response to penicillin. Methods: a 36-year-old Peruvian man, seropositive for HIV infection, was admitted to the hospital with decreased visual acuity in his right eye, hearing loss, tinnitus, buzzing, and vertigo. He referred history of syphilis in the previous two years. Results: oph-thalmological examination was performed. Ocular anterior segment examination of the right eye showed cells. Fundoscopy revealed swelling of the right optic disc with panuveitis. Diagnosis of neurosyphilis (NS) with optic and ear neuritis, and concurrent HIV infection was made, based on the clinical manifestations and serum and cerebrospinal VDRL titers. The patient was treated with intravenous penicillin (four million units every four hours for two weeks) with an excellent clinical response. Discussion: simultaneous optic and auditive cranial nerve involvement can manifest early neurosyphilis (NS) and HIV coinfection. This is the second report to describe the simultaneous occurrence of syphilitic optic neuritis with vestibulocochlear nerve involvement.

Aquaporin-4 protein antibody-associated optic neuritis related to neuroendocrine tumor after receiving an inactive COVID-19 vaccine

Neuromyelitis optica (NMO), also known as Devic’s disease, is a rare, autoimmune, and recurrent demyelinating disorder that primarily affects the spinal cord and optic nerve. We report a case with recurrent optic neuritis caused by the paraneoplastic NMO spectrum disorder in the setting of a gastric neuroendocrine tumor 2 weeks after receiving an inactive COVID?19 vaccine.

Disease course of Chronic Relapsing Inflammatory Optic Neuropathy (CRION) in a single care center / Evolución de la enfermedad de la neuropatía óptica inflamatoria crónica recidivante (CRION) en un solo centro de atención

ABSTRACT Background: Chronic relapsing inflammatory optic neuropathy (CRION) is a recurrent, idiopathic optic neuritis and is considered as a rare disease. Objective: To describe the clinical course during long-term follow-up of patients with a diagnosis of CRION. Methods: From a cohort of 1,735 patients with demyelinating disorders, we selected patients aged over 16 years with CRION according to current criteria. Demographic and clinical data, including initial presentation, symptoms, number of relapses, time delay in diagnosis, diagnostic methods, and treatment were obtained from clinical files. Infections, autoimmune diseases, and multiple sclerosis, among other conditions, were ruled out in all patients. Results: We analyzed 30 patients with CRION: 24 women and six men, with mean age of 42.8±10.2 years, median disease course of 7.9 years (5.29-13.1), and median number of attacks of 2 (IQR 2-4). The initial manifestation was ocular pain in 97% and bilateral and sequential affection in 87%. Visual acuity was recovered in 50%, did not improve in 33%, and recovered incompletely in 17%. Antibodies against aquaporin-4 (AQP4-Abs) were negative in 73.3%. Magnetic resonance imaging of the brain was normal in 76.7%. None of the patients evolved to another demyelinating disease over time. Initial treatment was methylprednisolone in 100%, and plasmapheresis in 20%. Currently, all patients are on maintenance treatment with mycophenolate mofetil or rituximab with a decrease in relapsing rate. Conclusions: Diagnosis of CRION is challenging and should be kept in mind. Prompt diagnosis, adequate treatment and close follow-up are essential to prevent disabling sequelae in these patients.

RESUMEN Antecedentes: Neuropatía óptica inflamatoria crónica recidivante (CRION) es una neuritis óptica idiopática recurrente, considerada una enfermedad rara. Objetivo: Describir la evolución clínica durante el seguimiento a largo plazo de pacientes con diagnóstico de CRION. Métodos: De una cohorte de 1.735 pacientes con trastornos desmielinizantes, seleccionamos pacientes mayores de 16 años con diagnóstico de CRION según los criterios actuales. Datos demográficos y clínicos, incluyendo presentación inicial, síntomas, recaídas, tiempo de retraso diagnóstico, métodos de diagnóstico y tratamiento se obtuvieron de los archivos clínicos. Se descartaron en todos los pacientes infecciones, enfermedades autoinmunes, esclerosis múltiple, entre otras condiciones. Resultados: Se analizaron 30 pacientes con diagnóstico de CRION: 24 mujeres y 6 hombres, edad media de 42,8±10,2 años, mediana del curso de la enfermedad de 7,9 años (5,2-13,1), mediana del número de recaídas 2 (IQR 2-4). La manifestación inicial fue dolor ocular en el 97% y afección bilateral y secuencial en el 87%. La agudeza visual mejoró en el 50%, sin recuperación en el 33% y con restauración incompleta en el 17%. Los anticuerpos contra acuaporina-4 (AQP4-Abs) fueron negativos en el 73,3%. La resonancia magnética cerebral fue normal en el 76,7%. Ningún paciente evolucionó hacia otra enfermedad desmielinizante en el seguimiento. El tratamiento inicial fue metilprednisolona en el 100%, y plasmaféresis en el 20%. Actualmente, todos los pacientes están en tratamiento de mantenimiento con micofenolato de mofetilo o rituximab con disminución de la tasa de recaídas. Conclusiones: El diagnóstico de CRION representa un desafío y debe tenerse en cuenta. El diagnóstico oportuno, tratamiento adecuado y seguimiento estrecho son fundamentales para evitar secuelas invalidantes.