Síndrome de Brown-Séquard como forma de presentación de mielitis transversa idiopática / Brown-Séquard syndrome as a presentation of idiopathic transverse myelitis

El síndrome de Brown-Séquard es el conjunto de signos y síntomas causado por hemisección medular de diversos orígenes. Puede generarse por múltiples causas; las traumáticas son las más frecuentes. Las causas menos frecuentes son patología inflamatoria, isquémica, tumoral o infecciosa. Se presenta un niño de 12 años, con instauración aguda y progresiva de un síndrome de hemisección medular derecho, con parálisis hipo/arrefléctica homolateral y afectación de sensibilidad termoalgésica contralateral. En la resonancia magnética de médula espinal, se observó compromiso inflamatorio en hemimédula derecha a nivel de segunda y tercera vértebras torácicas. Con diagnóstico de mielitis transversa idiopática, inició tratamiento con corticoide intravenoso a altas dosis con evolución clínica favorable y restitución de las funciones neurológicas.

Brown-Séquard syndrome refers to a set of signs and symptoms caused by hemisection of the spinal cord from various sources. It may have multiple causes; traumatic injuries are the most frequent ones. The less common causes include inflammation, ischemia, tumors, or infections. This report is about a 12-year-old boy with an acute and progressive course of right hemisection of the spinal cord, with ipsilateral hypo/areflexic paralysis and contralateral loss of thermalgesic sensation. The MRI of the spinal cord showed inflammation in the right side of the spinal cord at the level of the second and third thoracic vertebrae. The patient was diagnosed with idiopathic transverse myelitis and was started on intravenous high-dose corticosteroids; he showed a favorable clinical course and recovered neurological functions.

"Clock dial pattern", a radiologic clue to neuro-chikungunya diagnosis: a case series / "Padrão em ponteiro de relógio", uma dica radiológica para o diagnóstico de neurochikungunya: série de casos

Abstract Background Chikungunya is a mosquito-borne disease caused by the chikungunya virus (CHIKV) and can lead to neurological complications in severe cases. Objective This study examined neuroimaging patterns in chikungunya cases during two outbreaks in Brazil to identify specific patterns for diagnosis and treatment of neuro-chikungunya. Methods Eight patients with confirmed chikungunya and neurological involvement were included. Clinical examinations and MRI scans were performed, and findings were analyzed by neuroradiologists. Data on age, sex, neurological symptoms, diagnostic tests, MRI findings, and clinical outcomes were recorded. Results Patients showed different neuroimaging patterns. Six patients exhibited a "clock dial pattern" with hyperintense dotted lesions in the spinal cord periphery. One patient had thickening and enhancement of anterior nerve roots. Brain MRI revealed multiple hyperintense lesions in the white matter, particularly in the medulla oblongata, in six patients. One patient had a normal brain MRI. Conclusion The "clock dial pattern" observed in spinal cord MRI may be indicative of chikungunya-related nervous system lesions. Isolated involvement of spinal cord white matter in chikungunya can help differentiate it from other viral infections. Additionally, distinct brainstem involvement in chikungunya-associated encephalitis, particularly in the rostral region, sets it apart from other arboviral infections. Recognizing these neuroimaging patterns can contribute to early diagnosis and appropriate management of neuro-chikungunya.

Resumo Antecedentes A chikungunya é uma doença transmitida por mosquitos causada pelo vírus chikungunya (CHIKV) e pode levar a complicações neurológicas em casos graves. Objetivo Este estudo examinou padrões de neuroimagem em casos de chikungunya durante dois surtos no Brasil para identificar padrões específicos para o diagnóstico e tratamento de neurochikungunya. Métodos Oito pacientes com chikungunya confirmada e envolvimento neurológico foram incluídos. Exames clínicos e ressonâncias magnéticas (RM) foram realizados, e os achados foram analisados por neurorradiologistas. Dados sobre idade, sexo, sintomas neurológicos, testes diagnósticos, achados de RM e desfechos clínicos foram registrados. Resultados Os pacientes apresentaram diferentes padrões de neuroimagem. Seis pacientes apresentaram um "padrão de mostrador de relógio" com lesões pontilhadas hiperintensas na periferia da medula espinhal. Um paciente apresentou espessamento e realce das raízes nervosas anteriores. A RM do cérebro revelou múltiplas lesões hiperintensas na substância branca, especialmente no bulbo em seis pacientes. Um paciente apresentou uma RM cerebral normal. Conclusão O "padrão de mostrador de relógio" observado na RM da medula espinhal pode ser indicativo de lesões do sistema nervoso relacionadas à chikungunya. O envolvimento isolado da substância branca da medula espinhal na chikungunya pode ajudar a diferenciá-la de outras infecções virais. Além disso, o envolvimento distinto do tronco cerebral na encefalite associada à chikungunya, especialmente na região rostral, a distingue de outras infecções por arbovírus. O reconhecimento desses padrões de neuroimagem pode contribuir para o diagnóstico precoce e manejo adequado da neurochikungunya.

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.

Disautonomía gastrointestinal como manifestación de mielitis transversa: Reporte de caso

La mielitis transversa, de origen inflamatorio, es una afectación rara de la médula espinal que afecta a uno o varios niveles. La etiología incluye esclerosis múltiple, causas infecciosas o trastornos del espectro de la neuromielitis óptica. Se presenta de forma aguda, con síntomas motores, sensoriales y/o disautonómicos como los gastrointestinales y urinarios. El diagnóstico se basa en la sintomatología, evolución y se confirma por punción lumbar, resonancia magnética nuclear y analítica sanguínea completa. Se presenta el caso clínico de una paciente con mielitis transversa, que debutó con sintomatología gastrointestinal, síntomas motores y confirmación diagnóstica con resonancia magnética nuclear.

Inflammatory transverse myelitis is a rare condition that affects one or more levels of the spinal cord. Its etiology includes multiple sclerosis, infectious causes, or disorders within the spectrum of neuromyelitis optica. It presents acutely with motor, sensory, and/or dysautonomic symptoms, such as those related to the gastrointestinal and urinary systems. Diagnosis is based on symptomatology, evolution, and is confirmed by lumbar puncture, magnetic resonance imaging, and complete blood analysis. We present a clinical case of a patient with transverse myelitis who presented with gastrointestinal symptoms, motor symptoms, and was diagnosed with magnetic resonance imaging.

Mielitis transversa aguda asociada con infección por SARS-CoV-2 / Acute Transverse Myelitis Associated to SARS-CoV-2 Infection

Resumen Además de provocar problemas respiratorios, la infección con el SARS-CoV-2 puede causar un espectro amplio de complicaciones neurológicas como son cefalea, ageusia, anosmia, encefalopatía, enfermedad cerebrovascular, síndrome de Guillain-Barré y otras polineuropatías, meningoencefalitis, encefalopatía hemorrágica necrotizante aguda y síndromes del sistema nervioso central asociados a inflamación. En esta revisión presentamos 39 casos de mielitis transversa aguda (MTA) asociados a infección por el SARS-CoV-2, confirmado por una prueba de reacción en cadena de la polimerasa (PCR) por hisopado nasofaríngeo y/o de antígeno en plasma. Los análisis bioquímicos de los pacientes confirmaron la ausencia de otros patógenos y de autoanticuerpos involucrados en inflamación del sistema nervioso, excepto por 2 casos, uno con autoanticuerpos contra la glicoproteína de la mielina de los oligodendrocitos (anti-MOG IgG) y otro con anti-MOG IgG y antidescarboxilasa del ácido glutámico (anti-GAD65), indicativos de una enfermedad autoinmune del sistema nervioso central. Los estudios de imagenología de resonancia magnética (RMN) confirmaron el diagnóstico de MTA. Aunque no se puede inferir causalidad, es muy probable que los casos aislados de MTA sean consecuencia de un proceso para o postinfeccioso en la COVID-19. La comprensión de los mecanismos que desencadenan estos trastornos neurológicos durante o al final de la infección viral, ayudarán a optimizar las estrategias terapéuticas para el manejo del paciente.

Abstract In addition to causing respiratory problems, SARS-CoV-2 can cause a wide spectrum of neurological complications such as headaches, ageusia, anosmia, encephalopathy, cerebrovascular disease, Guillain-Barré syndrome and other polyneuropathies, meningoencephalitis, acute necrotizing hemorrhagic encephalopathy, and central nervous system syndromes associated with inflammation. In this review we present 39 cases of acute transverse myelitis (ATM) associated to SARS-CoV-2 infection. All cases had a positive polymerase chain reaction (PCR) nasopharyngeal swab and/or antigen test. Biochemical analyses confirmed the absence of other pathogens and autoantibody-mediated neuroinflammatory disease, except for two cases, one with autoantibodies against the oligodendrocyte myelin glycoprotein (anti-MOG IgG) and another with anti-MOG IgG and anti-glutamic acid decarboxylase (anti-GAD65). Magnetic Resonance Imaging (MRI) studies confirmed the diagnosis of ATM. Although causality cannot be inferred, it is likely that isolated cases of ATM are the consequence of a para or post-infectious process in SARS-CoV-2. In this work, the probable causes of ATM associated with SARS-CoV-2 are discussed. The understanding of the mechanisms behind these neurological disorders triggered by a viral infection will help to optimize the therapeutic strategies for patient management.

Mielopatía dorsal aguda como presentación atípica de una neurocisticercosis espinal

La neurocisticercosis espinal es una enfermedad infecciosa poco frecuente. Su presentación puede ser extraespinal o intraespinal y la mayoría de casos es de evolución subaguda o crónica. Se presenta el caso de una paciente mujer de 55 años, natural y procedente de Lima, Perú, con cuadro clínico de una paraparesia aguda secundaria a una mielopatía dorsal por lesiones quísticas de cisticercosis espinal. La paciente recibió tratamiento médico y quirúrgico con una evolución clínica y de imágenes favorable. Es importante considerar en nuestro contexto epidemiológico, la cisticercosis espinal como diagnóstico diferencial, ante un cuadro clínico de mielopatía aguda, ya que el adecuado enfoque diagnóstico y tratamiento oportuno de esta rara entidad pueden mejorar el pronóstico de los pacientes.

Spinal neurocysticercosis is an infectious and rare disease. Its presentation can be extraspinal or intraspinal and most cases are of subacute or chronic evolution. We report the case of a 55-year-old female patient from Lima, Peru with a 2-day history of acute paraparesis secondary to dorsal myelopathy due to cystic lesions of spinal cysticercosis. The patient received medical and surgical treatment with a favorable clinical and imaging evolution. In our epidemiological context, it is important to consider a spinal cysticercosis as a differential diagnosis when faced with a clinical picture of acute myelopathy, since the appropriate diagnostic approach and timely treatment of this rare entity can improve the prognosis of patients.

Neuromuscular Alterations Associated with COVID-19. A Scientific Literature Review

Introduction: The COVID-19 pandemic has brought short, medium, and long-term consequences on the quality of life of those affected. Among the complications are those related to the involvement of the nervous system and the structures involved in body movement, with sequelae that may be transitory and/or definitive, and require rehabilitation. Objective: Identify the neuromuscular alterations that affect body movement, associated with COVID-19. Material and methods: A search was made for observational works published in the SCOPUS, PubMed, EBSCO, and Nature databases between January 2020 and June 2022 under the PRISMA methodology, to answer the PICO question: what are the neuromuscular alterations that can potentially affect movement, associated with COVID-19? The established filters were type of study, language, age, availability, publication dates. The MeSH terms were SARS-Cov-2, COVID-19, Long Covid, Motor Activity Neuromuscular Diseases, Neurological Disorders, Guillain-Barré Syndrome, Myelitis Transverse, Stroke, Patient, Peripheral Nervous System Diseases. The methodological quality was evaluated according to STROBE and the level of evidence was established according to CEBM. Results: In the first search, 645 articles were identified. 637 were discarded by filters, titles, duplicate abstracts, methodological quality, and level of evidence. There were 8 articles selected for the present review in which neuromuscular alterations of central and peripheral origin were identified, such as myalgias, fatigue, polyneuroradiculopathies, CNS inflammation, among others, with clinical manifestations that affect movement. Conclusion: COVID-19 is a multisystemic disease that can affect the nervous system with symptoms of neuromuscular alterations that compromise body movement.

Introducción: La pandemia por la COVID-19 ha traído consecuencias a corto, mediano y largo plazo sobre la calidad de vida de los afectados. Entre las complicaciones se encuentran aquellas relacionadas con la afectación del sistema nervioso y las estructuras involucradas en el movimiento corporal, con secuelas que pueden ser transitorias y/o definitivas, y requieren rehabilitación Objetivo: Identificar las alteraciones neuromusculares que afectan el movimiento corporal, asociadas a la COVID-19. Material y métodos: Se hizo una búsqueda de trabajos observacionales publicados en las bases de datos SCOPUS, PubMed, EBSCO y Nature entre enero de 2020 y junio de 2022 bajo metodología PRISMA, para dar respuesta a la pregunta PICO: ¿cuáles son las alteraciones neuromusculares que potencialmente pueden afectar el movimiento, asociadas a la COVID-19? Los filtros establecidos fueron tipo de estudio, idioma, edad, disponibilidad y fechas de publicación. Los términos MesH fueron SARS-Cov-2, COVID-19, Long Covid, Motor Activity Neuromuscular Diseases, Neurological Disorders, Guillain-Barré Syndrome, Myelitis Transverse, Stroke, Patient, Peripheral Nervous System Diseases. La calidad metodológica se evaluó según STROBE y el nivel de evidencia se estableció según CEBM. Resultados: En la primera búsqueda se identificaron 645 artículos. Posteriormente se descartaron 637 por filtros, títulos, resúmenes duplicados, calidad metodológica y nivel de evidencia. Así, quedaron seleccionados 8 para la presente revisión, en los cuales se identificaron alteraciones neuromusculares de origen central y periférico, como mialgias, fatiga, polineuroradiculopatías, inflamación del SNC, entre otras, con manifestaciones clínicas que afectan el movimiento. Conclusión: La COVID-19 es una enfermedad multisistémica que puede afectar el sistema nervioso con síntomas de alteraciones neuromusculares que comprometen el movimiento corporal.

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.

Mielopatias inflamatórias / Inflammatory myelopathies / Mielopatías inflamatorias

Mielopatia inflamatória ou mielite transversa é uma síndrome neurológica potencialmente incapacitante com uma variedade de etiologias. Episódios únicos ou recorrentes podem resultar em dependência de cadeira de rodas. O quadro clínico de fraqueza, alteração de sensibilidade e disfunção autonômica de início agudo ou subagudo é marca dessa síndrome. Esse cenário é comum às diferentes etiologias, que podem ser de natureza desmielinizante, por doença autoimune sistêmica, paraneoplásica ou infecciosa. A ressonância magnética de coluna é o exame de neuroimagem de escolha. Exames complementares como avaliação do líquido cefalorraquidiano, testes sorológicos e pesquisa de anticorpos dão suporte à investigação. A depender da etiologia, há tratamentos específicos a fim de reduzir incapacidade e chance de novos surtos, além de diferentes prognósticos. Este trabalho objetiva uma revisão de literatura sobre mielopatias inflamatórias e suas principais etiologias, a partir de dados obtidos na plataforma eletrônica PubMed. Para a discussão, foram revisadas as etiologias desmielinizantes (encefalomielite disseminada aguda, esclerose múltipla, doença do espectro, neuromielite óptica e neurite óptica, encefalite e mielite associadas ao MOG-IgG); doenças autoimunes (lúpus eritematoso sistêmico e síndrome de Sjögren); síndromes paraneoplásicas e mielopatias infecciosas (neuroesquistossomose, mielite por HIV e por HTLV-1 e neurossífilis). Concluiu-se com este estudo que a mielopatia inflamatória é uma condição de gravidade variável que produz potencial incapacidade, causada por diferentes etiologias, porém com quadro clínico comum entre elas. Por isso, é importante conhecer cada uma dessas causas, a fim de promover o melhor e mais precoce tratamento e reduzir sequelas.

Inflammatory myelopathy or transverse myelitis is a potentially disabling neurological syndrome with various etiologies. Single or recurrent episodes can result in wheelchair dependence. A clinical picture of weakness, altered sensitivity, and autonomic dysfunction with acute or subacute onset is characteristic of this syndrome. This scenario is common to different etiologies, which can be of a demyelinating nature, due to systemic, paraneoplastic, or infectious autoimmune disease. Spine MRI is the neuroimaging test of choice. Complementary tests such as cerebrospinal fluid evaluation, serological tests and antibody research support the investigation. Depending on the etiology, there are specific treatments to reduce disability and the chance of new episodes, and different prognoses. This study is a literature review on inflammatory myelopathies and their main etiologies, based on data obtained from the PubMed database. Demyelinating etiologies (acute disseminated encephalomyelitis, multiple sclerosis, neuromyelitis optic spectrum disease and optic neuritis, MOG-IgG-associated encephalitis and myelitis), autoimmune diseases (systemic lupus erythematosus and Sjögren's syndrome), paraneoplastic syndromes and infectious myelopathies (neuroschistosomiasis, HIV and HTLV-1 myelitis, and neurosyphilis) were reviewed for discussion. In conclusion, inflammatory myelopathy is a condition of variable severity that produces potential disability, caused by different etiologies, but with a common clinical picture between them. Thus, knowledge on each of these causes is important to promote the best and earliest treatment and reduce sequelae.

La mielopatía inflamatoria o mielitis transversa es un síndrome neurológico potencialmente incapacitante con una variedad de etiologías. Los episodios únicos o recurrentes pueden tener como consecuencia dependencia de silla de ruedas. El cuadro clínico de debilidad, sensibilidad alterada y disfunción autonómica de inicio agudo o subagudo es distintivo de este síndrome. Esto es común a diferentes etiologías, que pueden ser de naturaleza desmielinizante, debido a enfermedades autoinmunes sistémicas, paraneoplásicas o infecciosas. La resonancia magnética de columna es la prueba de neuroimagen de elección. Las pruebas complementarias, como la evaluación del líquido cefalorraquídeo, las pruebas serológicas y la investigación de anticuerpos respaldan la investigación. Dependiendo de la etiología, existen tratamientos específicos para reducir la discapacidad y la posibilidad de nuevos brotes, además de diferentes pronósticos. Este trabajo tiene como objetivo revisar la literatura sobre mielopatías inflamatorias y sus principales etiologías desde los datos obtenidos de la base de datos electrónica PubMed. Se revisaron las etiologías desmielinizantes (encefalomielitis aguda diseminada, esclerosis múltiple, enfermedad del espectro, neuromielitis óptico y neuritis óptica, encefalitis y mielitis asociadas a MOG-IgG), las enfermedades autoinmunes (lupus eritematoso sistémico y síndrome de Sjögren), los síndromes paraneoplásicos y mielopatías infecciosas (neurosquistosomiasis, mielitis por VIH y HTLV-1 y neurosífilis). Se concluyó que la mielopatía inflamatoria es una condición de severidad variable, que produce potencial discapacidad causada por diferentes etiologías, pero tiene un cuadro clínico común entre ellas. Por ello, es importante conocer cada una de las causas para promover el mejor y más precoz tratamiento, además de reducir las secuelas.

COVID-19 associated myelitis:case series

The coronavirus pandemic brought with it a wide range of clinical presentations. Earlier, the respiratory symptoms comprised most of the clinical picture. However, as more and more people got infected, many atypical presentations came into the limelight, especially the neurological manifestations. Spinal cord complications are widely reported, with COVID-19 associated myelitis constituting a big part. Through this report, we bring you a series of cases of COVID-19 associated myelitis to add to the already available data. We report four patients, two of whom developed longitudinally extensive myelitis (three or more vertebral segments). The other two suffered from multisegmented short-segment myelitis (less than three vertebral segments). COVID-19 myelitis can be seen during COVID-19 illness and post COVID. We aim to familiarize the medical community with this entity so that there is a minimum delay between the onset of the symptoms in the patient and the management of this complication, as the treatment is often gratifying.

Japanese encephalitis presenting as transverse myelitis: An uncommon presentation

Japanese Encephalitis Virus (JEV) is the main cause of viral encephalitis in South East Asia. Commonly, it presents as an acute encephalitic syndrome with fever, headache, seizures, and altered sensorium as clinical manifestations. However, there can be atypical presentations such as acute transverse myelitis (ATM) as the initial manifestation. Clinicians should be aware of such possibilities and myelitis due to the JE virus should be considered as a differential in children presenting with encephalomyelitis

TRANSVERSE MYELITIS DUE TO TUBERCULOSIS: CASE REPORT WITH NARRATIVE REVIEW

Tuberculosis is a global public health problem, which continues to increase morbidity and mortality around the world. The infectious agent is the well-known Koch bacillus, Mycobacterium tuberculosis complex, discovered in 1882 by Robert Koch. Tuberculosis in the central nervous system with spinal cord involvement is a rare event. It is believed that the medullar infection is due to the contiguity of a bone infection at the vertebral level. The initial symptoms of spinal cord infection are due to the motor and sensory compromise of the compromised spinal cord level. Infection in the central nervous system occurs mainly due to the presentation of meningitis and intramedullary tuberculosis in HIV-positive patients, which is considered to represent only 2% of the cases of tuberculosis of the central nervous system. We present a case of spinal cord involvement due to tuberculosis.

Clinico-epidemiologic characteristics of optic neuritis in a tertiary eye centre in Eastern India based on the status of serum aquaporin-4 antibody

Purpose: To elucidate the clinico?epidemiologic characteristics of optic neuritis based on the status of serum aquaporin?4 antibody (AQP4?Ab) in patients with optic neuritis (ON). Methods: Medical records of 106 patients with ON and a follow?up of 3 years were reviewed. For each patient, the following data were extracted: medical history, findings of the ocular examination, brain, orbital or spinal MRI, and serological tests for AQP4. The ON was classified as typical or atypical based on disc examination and improvement in vision after intravenous methylprednisolone (IVMP). The clinical findings (typical or atypical), disease course, and outcomes were analyzed according to the serostatus of the ON. Results: 10 patients ((9.4%) were seropositive for AQP4?Ab; all had atypical ON. 96 patients (91%) were seronegative for AQP4?Ab: 36 atypical ON and 60 typical ON. Profound visual impairment at presentation was seen in all patients. However, at the end of the study period, seropositive and seronegative atypical ON had poor visual outcomes as compared to seronegative typical ON (P = 0.002). Five seropositive and four seronegative patients with atypical ON developed transverse myelitis. Bilateral disease with relapse was more in seropositive patients (80%); however, seronegative with atypical ON also had bilateral presentation and relapse in 42% and 41%, respectively. Conclusion: AQP4?Ab seropositive patients mostly present with atypical features such as bilateral recurrent ON, poor visual outcome, and increased incidence of transverse myelitis. However, atypical clinical features can also be seen in seronegative ON with a poor visual outcome and a recalcitrant course.

Mielite transversa por Zika vírus em paciente com Lúpus Eritematoso Sistêmico (LES) - relato de caso em Manaus - AM / Transverse myelitis by Zika virus in patient with Systemic Lupus Erythematosus (SLE) - case report in Manaus - AM

Objetivo: Relatar caso clinico de uma paciente com Mielite Transversa diagnosticada com Lúpus Eritematoso Sistêmico e Zika Vírus. Relato de Caso: Paciente diagnosticada em Lúpus Eritematoso Sistêmico (LES) com nefrite lúpica classe IV há 15 anos em remissão, iniciou quadro de mialgia difusa prejudicando deambulação. Após melhora espontânea do quadro, paciente permaneceu com queixa de astenia e cefaleia intensa unilateral esquerda recorrente, evoluindo com síncope, paraparesia em membro inferior em caráter progressivo ascendente seguido de crise convulsiva tipo tônico clônico generalizado de inicio disruptivo. Um primeiro exame do liquor cefalorraquidiano (LCR) foi solicitado, sem evidência de alteração na analise bioquímica simples. Outras alterações laboratoriais foram identificadas, além do método de reação em cadeia de polimerase (PCR) para Zika vírus na urina e liquor cefalorraquqidiano (após recoleta) detectáveis. Foram detectadas alterações à ressonância magnética compatíveis com mielite transversa. A investigação etiológica durou dois meses e meio, com duas internações no período. Apesar das manifestações neurológicas do Zika Vírus serem ordinariamente inespecíficas, raras e brandas, não se deve desconsiderar a hipótese diagnostica de mielite transversa em área endêmica para arboviroses com manifestações neurológicas mesmo após liquor cefalorraquidiano e outros exames inespecíficos com o achado clínico, considerando paciente remissiva do quadro de lupus eritematoso sistêmico há 15 anos

Objective: case report of a patient with transverse myelitis diagnosed with systemic lupus erythematosus and zika Virus. Case report: Patient diagnosed with Systemic Lupus Erythematosus (SLE) with class IV lupus nephritis for 15 years in remission, begins to have diffuse myalgia, impairing walking. After spontaneous improvement of the condition, the patient remained complaining of asthenia and recurrent left unilateral severe headache, evolving with syncope, progressive ascending paraparesis in the lower limb, followed by a generalized tonic clonic seizure type of disruptive onset. A first examination of the cerebrospinal fluid (CSF) was requested, without evidence of alteration in the simple biochemical analysis. Other laboratory alterations were identified, in addition to the polymerase chain reaction (PCR) for detectable Zika virus in urine and cerebrospinal liquor (after collection). Changes were detected on magnetic resonance imaging (MRI) compatible with transverse myelitis. The etiological investigation lasted two and a half months, with two hospitalizations in the period. Although the neurological manifestations of Zika Virus are ordinarily nonspecific, rare and mild, the diagnostic hypothesis of transverse myelitis in an endemic area for arboviruses with neurological manifestations should not be disregarded even after cerebrospinal fluid and unspecific with the other clinical, remissive of the clinical picture lupus erythemasous systemic 15 years ago.

Relación entre mielitis transversa y vacunas: actualización y revisión de la literatura

La mielitis transversa (MT) es un síndrome clínico caracterizado por una inflamación y desmielinización aguda de la médula espinal. Esta entidad tomó relevancia pública en septiembre de 2020 cuando fueron suspendidos los ensayos clínicos de la vacuna ChAdOx1 para SARS-CoV-2, desarrollada por AstraZeneca y la Universidad de Oxford, por casos de MT en tres participantes. Al respecto, esta investigación se plantea recopilar la información disponible que relacione casos de MT con vacunas a nivel mundial. En este sentido, se llevó a cabo una investigación descriptiva, con diseño documental, donde se efectuó la revisión sistemática de publicaciones disponibles en las bases de datos: "PubMed", "Redalyc" y "SciELO", desde el 01/2000 al 04/2021, que presentan casos clínicos de MT post vacunación. Se estudiaron 49 casos de MT posterior a la vacunación contra diferentes virus y bacterias, 81,8 % de los mismos sucedieron en el primer mes. Se argumenta que los antígenos de las vacunas o sus adyuvantes pueden propiciar complicaciones autoinmunes que explican este fenómeno. Sin embargo, los análisis estadísticos no encuentran relación causal clara, mientras que existe evidencia que sugiere que las vacunas pueden contribuir a que se manifieste un trastorno autoinmune subyacente. El riesgo de MT pos-vacunación parece ser muy pequeño o depender del azar, mientras que el beneficio derivado de la vacunación está demostrado, incluso para disminuir los trastornos autoinmunitarios. No obstante, es importante notificar y estudiar estos casos para la realización de estimaciones certeras en el futuro, contribuyendo al desarrollo de la medicina de precisión y personalizada

Transverse myelitis (TM) is a clinical syndrome characterized by acute inflammation and demyelination of the spinal cord. This entity gained public relevance in September 2020 when clinical trials of the ChAdOx1 vaccine for SARS-CoV-2, developed by AstraZeneca and the University of Oxford, were suspended due to possible cases of MT in three participants. For this reason, this research aims to compile the available information that relates MT cases to vaccinations worldwide. In this sense, a descriptive research with documentary design was carried out through a systematic review of the publications available in the databases "PubMed", "Redalyc" y "SciELO" that presented clinical cases of post-vaccination TM from 01/2000 to 04/2021. We studied 49 cases of post-vaccination MT for different viruses and bacteria, 81.8 % of which occurred in the first month. In this sense, It's argued that vaccine antigens or their adjuvants may promote autoimmune complications that explain this phenomenon. However, statistical analyses find no certain causal relationship, while there is evidence to suggest that vaccines may contribute to the development of an underlying autoimmune disorder. The risk of postvaccination MT appears to be very small or dependent on chance, whereas the benefit derived from vaccination is certain, including in decreasing autoimmune disorders. However, it is important to report and study these cases for accurate estimates in the future, contributing to the development of precise and personalized medicine

Síndrome antifosfolípido no trombótico: manifestaciones inflamatorias hematológicas y neurológicas / Non thrombotic antiphospholipid syndrome: neurologic and hematologic inflammatory manifestations

RESUMEN El síndrome antifosfolípido (SAF) es una entidad caracterizada por la presencia de anticuerpos antifosfolípidos y características clínicas como la pérdida fetal recurrente y la trombosis venosa o arterial. El diagnóstico del SAF se realiza extrapolando criterios de clasificación que incluyen criterios clínicos y de laboratorio, con manifestaciones trombóticas en su gran mayoría. Sin embargo, hay una gran variedad de manifestaciones no trombóticas de la enfermedad no incluidas en los criterios de clasificación. Dichas manifestaciones "extra-criterio" son aquellas que, debido a su frecuencia y a la relevancia que tienen para la toma de decisiones, son hallazgos de valor. Entre ellas, destacan características inflamatorias en dominios como el hematológico, de las cuales la principal es la trombocitopenia. De las diferentes manifestaciones neurológicas, debido a su frecuencia o gravedad, resaltan la migraña, la epilepsia, la demencia y otras graves como la corea y la mielitis. En la presente revisión se reseña la información disponible sobre las manifestaciones de dos dominios frecuentes y relevantes "extra-criterio" del síndrome antifosfolípido: las hematológicas y las neurológicas.

SUMMARY Antiphospholipid syndrome (APS) is an entity defined for the presence of antiphospholipid antibodies and events such as recurrent fetal loss and arterial or venous thrombosis. APS diagnosis is made through classification criteria, including laboratory criteria and clinical manifestations (most of them being thrombotic manifestations). Nonetheless, there is a wide variety of non-thrombotic manifestations of APS, which are not included in classification criteria. These "noncriteria" manifestations are important for decision making, based on their frequency or clinical relevance. Among them, some domains stand out: the hematologic domain, being thrombocytopenia the main manifestation; and the neurological domain with different manifestations such as migraine, epilepsy, dementia and severe manifestations, such as chorea and myelitis. Therefore, in the present article, information concerning two of the most common "non-criteria" domains (neurological and hematological) of APS is reviewed.

Scorpion Bite Atypically Presenting As Acute Transverse Myelitis And Sub Arachnoid Hemorrhage: A Case Report And Review

Scorpion bite is an important health issue as it has been reportedthatabout tenpersons are killed by a venomous scorpion for each killed by a venomous snake.Scorpion venom may be cardiotoxic, hemotoxic, nephrotoxic or even neurotoxic. It acts on the autonomic nervous system producing parasympathetic and sympathetic manifestations.However, few have reported sub arachnoid haemorrhage and transverse myelitis occurring due to scorpion venom.Case Report:We are reporting a case of 50year old male who prese nted three days after an episode of scorpion bitewith paraplegia and inability to pass urine and stool due to transverse myelitis and subarachnoid hemorrhage. He was investigatedand treated accordingly. Clinical improvement was seen within tendays after the initiation of therapy. Conclusion:Scorpion sting,though rarely may present as SAH and transverse myelitis which are reversible and easily treatable.Clinical Significance:As scorpion bite is treatable,having high index of suspicion for scorpion sting inpatients of SAH and acute transverse myelitis in whom the cause of their clinical features could not be recognised may help in improving the outcome considerably in these cases.

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.

Longitudinal Extensive Transverse Myelitis as a Neurological Sequelae post-Sea Urchin Stings: A Case Report

@#Puncture injury from sea-urchin stings may lead to a local and systemic inflammatory reaction. We are reporting a case of longitudinal extensive transverse myelitis (LETM), which occurred ten days post-sea-urchin stings, where the patient presented with bilateral lower limb weakness. MRI showed multilevel segment spinal cord T2-weighted hyperintensity. Prompt intravenous methylprednisolone was administered, and the patient had a full recovery. To date, there is no case report of LETM associated with sea-urchin stings. Possible mechanism due to delayed immunological hypersensitivity to sea-urchin venom. This case demonstrates the potential serious neurological sequelae that may be associated with post-sea-urchin sting and the importance of prompt recognition and management in aiding recovery.