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.

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.

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

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.

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

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.

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.

Mielitis longitudinal extensa de presentación muy tardía: Una entidad del espectro de la neuromielitis óptica / Extensive longitudinal myelitis of very late presentation. An entity of the spectrum of optic neuromyelitis

Las enfermedades del espectro neuromielitis óptica son trastornos inflamatorios del sistema nervioso central caracterizados por una grave desmielinización y daño axonal inmunomediado que afecta principalmente a los nervios ópticos y médula espinal. Suelen presentars e en edades tempranas, aunque existen algunas comunicaciones en la literatura de pacientes con presentaciones tardías. Presentamos el caso de una mujer de 78 años que consultó por un cuadro de paraparesia grave, trastornos sensitivos y retención urinaria. Se realizó una resonancia magnética de columna cervicodorsal que evidenció una lesión medular longitudinal extensa. Se descartaron otras causas secundarias, basadas en la clínica y en resultados de laboratorio. El dosaje de anticuerpos anti-acuaporina 4 resultó positivo. Se indicó tratamiento con glucocorticoides a altas dosis y plasmaféresis, y mantenimiento con rituximab, obteniendo escasa respuesta clínica. En pacientes con lesiones medulares extensas se deben contemplar múltiples diagnósticos diferenciales según la presentación clínica, hallazgos mediante estudios por imágenes y epidemiología. Asimismo, debe incluir la búsqueda de anticuerpos anti-acuaporina 4 y contra la glicoproteína de la mielina del oligodendrocito, ya que el pronóstico funcional de estos pacientes suele ser desfavorable debido al gran componente destructivo de las lesiones. En consecuencia, el tratamiento temprano es fundamental a fin de limitar el daño agudo y prevenir futuras recaídas, lo cual es especialmente importante en presentaciones tardías de esta entidad debido a la escasa reserva funcional y baja capacidad de remielinización.

Optic neuromyelitis spectrum diseases are inflammatory disorders of the central nervous system characterized by severe demyelination and immunomediated axonal damage that mainly affects the optic nerves and spinal cord. They usually appear at an early age, although there are some reports in the literature of patients with late presentations. We present the case of a 78-year-old woman who consulted for severe paraparesis, sensory disorders, and urinary retention. An MRI of the cervicodorsal spine was performed, showing extensive longitudinal spinal injury. Secondary causes based on clinical observations and laboratory studies were ruled out. The dosage of anti-aquaporin 4 antibodies was positive. Acute treatment with high-dose glucocorticoids and plasmapheresis was indicated, and maintenance with rituximab, obtaining little clinical response. In patients with extensive spinal injuries, multiple differential diagnoses should be considered according to the clinical presentation, findings through imaging studies and epidemiology. Likewise, it should include the search for anti-aquaporin 4 antibodies and against the oligodendrocyte myelin glycoprotein, since the functional prognosis of these patients is usually unfavourable due to the large destructive component of the lesions. Consequently, early treatment is essential in order to limit acute damage and prevent future relapses, which is especially important in late presentations of this entity due to the low functional reserve and low remyelination capacity.

Clinical and treatment patterns in neuromyelitis optica: a retrospective study from a single centre

Background: The aim of the study was to study the different presentations, treatment patterns and relapses on therapy in patients of neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorder (NMOSD).Methods: This is a retrospective, observational study in a tertiary hospital where Demographics, clinical manifestations at onset and at follow up and relapses, serum anti Aquaporin 4 antibody status, first line immunomodulatory therapy which was initiated and Relapses on first line therapy were noted.Results: Demographics and clinical presentation was largely similar to published data. 80% patients presented with LETM/ON at onset. Ten patients relapsed on oral therapy and trend was to shift from oral therapy to RTX after relapse on oral agent. No relapses were noted on RTX.Conclusions: Unaffordability and apprehension towards injections and cost were the factors affecting IMT decision, so majority received oral agents Aza/ MMF as first line therapy while remaining patients on oral therapy remained relapse free.

Mielitis Transversa Análisis Clínico y Revisión de Caso / Transverse Myelitis, Clinical Analysis and Case Review

Resumen: La mielitis transversa es una enfermedad inflamatoria y desmielinizante aguda o subaguda que se desarrolla en ausencia de afectación neurológica previa y compromete las vías sensitiva y motora además del control autónomo de la medula espinal. El cuadro se presenta como un dolor lumbar localizado, parestesias de inicio súbito en miembros inferiores con pérdida de la sensibilidad y paraparesia que puede evolucionar a paraplejia a lo que se suma comúnmente la disfunción vesical e intestinal; la coordinación y la sensibilidad de los miembros superiores también puede verse comprometida La incidencia de esta patología es baja, en los Estados Unidos oscila entre 14.000 casos nuevos en el año, de los cuales 33.000 persisten con secuelas. Afecta a hombres, mujeres y niños de todas las razas por igual, presentándose con más frecuencia entre los 10-19 años y los 30-39 años de edad. El diagnóstico diferencial incluye: síndrome de Guillain-Barré, compresión medular por tumores, mielopatías de origen vascular, esclerosis múltiple, neuromielitis óptica entre otros. El diagnóstico se basa en la presunción clínica, ante la cual debe solicitarse una resonancia magnética de manera urgente. El siguiente paso es realizar una punción lumbar para estudio en líquido cefalorraaquídeo (LCR) de células blancas, IgG y albúmina. El tratamiento de la mielitis transversa va encaminado hacia la resolución del proceso inflamatorio a nivel medular y la detención del avance del mismo.

Abstract Transverse myelitis is an acute or subacute inflammatory demyelinating disease that develops in the absence of previous neurological involvement and compromises the sensory and motor pathways in addition to the autonomous control of the spinal cord, the table is presented as a localized back pain, sudden paresthesia in lower limbs with loss of sensation and paraparesis which may progress to paraplegia commonly associated with bladder and bowel dysfunction; coordination and sensitivity of the upper limbs may also be compromised The incidence of this disease is low, in the United States ranges from 14,000 new cases per year of which 33,000 remain with sequelae. It affects men, women and children of all races equally predominantly between 10-19 years and 30-39 years of age. Differential diagnoses include Guillain- Barré syndrome, compression by spinal cord tumors, vascular myelopathy, multiple sclerosis, neuromyelitis óptica spectrum diseases amongst others. Diagnosis is based on clinical suspicion upon which an MRI should be requested urgently, the next step is to perform a lumbar puncture to study CSF white cell count, IgG and albumin. The treatment of transverse myelitis is aimed towards resolution of the inflammatory process in the spinal cord and avoiding neural deficit progression.

Potential role of dengue virus, chikungunya virus and Zika virus in neurological diseases

This study showed that laboratory markers of recent infection by dengue, Zika or chikungunya arboviruses were detected in the biological samples of approximately one-third of patients with encephalitis, myelitis, encephalomyelitis or Guillain-Barré syndrome, in a surveillance programme in Piauí state, Brazil, between 2015-2016. Fever and myalgia had been associated with these cases. Since in non-tropical countries most infections or parainfectious diseases associated with the nervous system are attributed to herpesviruses, enteroviruses, and Campylobacter jejuni, the present findings indicate that in tropical countries, arboviruses may now play a more important role and reinforce the need for their surveillance and systematic investigation in the tropics.

Role of microRNA-92b in the pathophysiology of acute transverse myelitis in children / 中华实用儿科临床杂志

Objective To explore microRNAs that play key regulatory roles in the pathophysiology of acute transverse myelitis in children,and to find therapeutic targets.Methods Twelve patients with acute transverse myelitis were enrolled as ATM group and three children with normal cerebrospinal fluid as the control group.MicroRNA in cerebrospinal fluid of children with acute transverse myelitis was detected by using microarray4.0 chip.Bioinformatics was used to demonstrate microRNA,which plays a key regulatory role,and to predict target genes.Real-time quantitative polymerase chain reaction (qPCR) technique was adopted for in biology and technology duplication.Enzyme-linked immunosorbent assay (ELISA) and Western blot technique were used to detect the expression of key miRNA target protein.The key candidate microRNA was inhibited/overexpressed in dorsal root ganglion neurons,and the function was verified in vitro.Flavopiridol was used to inhibit the activity of CDKs to verify that miR-92b worked through p57-CDKs-GAP-43 pathway.Results The characteristic elevation of miR-92b in cerebrospinal fluid samples of acute transverse myelitis was significant.Bioinformatics analysis showed that p57 was the target gene of miR-92b.The expression of miR-92b was contrary to the p57 protein.In vitro experiments showed that the length of axons in miR-92bmimics group was significantly shorter than that in the blank group.The axons of neurons in antimiR-92b group were obviously prolonged.In the miR-92b mimics + Flavopiridol groups,the axons of neurons were still significantly prolonged compared with that in the blank group.Western blot showed that p57 and GAP-43 protein expression in miR-92b mimics group was lower than that in blank group.The expression of p57 and GAP-43 protein in antimiR-92b group was significantly higher than that in blank group.But in miR-92b mimics + Flavopiridol group,the expression of p57 was lower compared with that in blank group,and the expression of GAP-43 protein was higher compared with that in blank group.Conclusions Up-regulation of miR-92b in children with acute transverse myelitis leads to a down-regulation of p57.The activity of CDKs is enhanced,which inhibits the expression of GAP-43 protein and the regeneration of axons in spinal cord injury region.MiR-92b is one of the key targets in the treatment of children with acute transverse myelitis.

Mielitis transversa asociada con infección por virus de chikunguña / Transverse myelitis associated to chikungunya virus infection

Resumen El virus de chikunguña se manifiesta en los seres humanos como una tríada de síntomas: fiebre, erupción cutánea y artralgias. El nombre de chikunguña se derivó del debilitante dolor en las articulaciones en las poblaciones africanas durante un brote en 1952-1953, en lo que ahora se conoce como Tanzania. La palabra local chikunguña significa lo que dobla hacia arriba, como resultado de la postura encorvada causada por el dolor que causa la enfermedad. La infección por el virus de chikunguña normalmente es una enfermedad de alivio espontáneo y puede manifestarse como encefalopatía, encefalitis, miocarditis, hepatitis e insuficiencia multiorgánica; sin embargo, se han reportado pocos casos con afección del sistema nervioso central, con mortalidad de 10% en este grupo de pacientes. Entre las complicaciones neurológicas reportadas están las encefalitis, miopatías, neuropatías y polineuropatía.

Abstract Chikungunya virus is presented as a triad of symptoms: fever, rash and joint pain in humans. Chikungunya name was derived from debilitating joint pain in African populations during an outbreak in 1952-1953, in what is now known as Tanzania. The local word chikungunya means that which bends up as a result of the stooped posture secondary to pain caused by the disease. Infection due to chikungunya virus is usually a self-limiting disease. It may manifest as encephalopathy, encephalitis, myocarditis, hepatitis and multiple organ failure, but few cases have been reported with the central nervous system disease with a mortality of 10% in this group of patients. The neurological complications reported include encephalitis, myopathy, neuropathy and polyneuropathy.

Caracterización de la mielitis transversa en un hospital referencia del sur colombiano / Characterization of patients with transverse myelitis in a referral hospital in southern Colombia

Objetivo: caracterizar a los pacientes con diagnóstico de mielitis transversa (MT) en un hospital de referencia del sur de Colombia durante los años 2007 a 2013. Materiales y métodos: Estudio transversal. Se incluyeron todos los pacientes con diagnóstico de MT, según los criterios The Transverse Myelitis Consortium Working Group. Resultados: En total 21 casos de MT (1,3 casos/10 000 ingresos), el 66,7% en edades entre 15-30 años, 57,1% de género femenino; 81,0% agudos. Todos presentaron déficit motor, 52,4% disfunción vesical autonómica, 81,0% nivel sensitivo. Se documentaron hallazgos imagenológicos de MT en resonancia magnética nuclear en el 71,4% de los casos e hiperproteinorraquia en 50%. En el 66,7% la etiología fue desconocida, en los casos en quienes se logró determinar la etiología fue: herpes simple tipo 1 (9,5%), varicela zoster (9,5%), leucemia mieloide aguda (4,8%), lupus eritematoso sistémico (4,8%) y deficiencia de vitamina B12 (4,8%). Conclusiones: La MT es un importante problema de salud pública, es necesario considerar sus características clínicas, establecer las formas idiopáticas y reconocer etiologías infecciosas dado el impacto terapeutico y pronóstico

Objective: To characterize patients with a diagnosis of transverse myelitis (TM) in a referral hospital in southern Colombia during years 2007 to 2013. Material and methods: This is a crosssectional study. All patients with a TM diagnosis were included, according to the criteria set by the Transverse Myelitis Consortium Working Group. Results: We had a total number of 21 cases of TM (1.3 cases/10,000 admissions). Two thirds (66,7%) of all patients were between 15-30 years of age, 57,1% were female; 81.0% had an acute presentation, 100% had motor deficit, 52.4% had autonomic bladder dysfunction, and 81.0% had sensitive level. MT findings were documented using magnetic resonance imaging in 71.4% of cases and 50% by high protein levels in the cerebrospinal fluid. Two thirds (66.7%) of the cases had an unknown etiology. In those cases in which their origin could be identified, the etiologies were: Type I Herpes Simplex (9.5%); Varicella Zoster (9.5%), acute myeloid leukemia (4.8%), systemic lupus erythematosus (4.8%) and vitamin B12 deficiency. Conclusion: MT is a major public health problem, and it is necessary to consider its clinical features, to establish its idiopathic forms and to recognize its infectious causes given their therapy impact and prognosis

Assessment of acute motor deficit in the pediatric emergency room / Avaliação do déficit motor agudo no ambiente de pronto socorro pediátrico

Abstract Objectives: This review article aimed to present a clinical approach, emphasizing the diagnostic investigation, to children and adolescents who present in the emergency room with acute-onset muscle weakness. Sources: A systematic search was performed in PubMed database during April and May 2017, using the following search terms in various combinations: "acute," "weakness," "motor deficit," "flaccid paralysis," "child," "pediatric," and "emergency". The articles chosen for this review were published over the past ten years, from 1997 through 2017. This study assessed the pediatric age range, from 0 to 18 years. Summary of the data: Acute motor deficit is a fairly common presentation in the pediatric emergency room. Patients may be categorized as having localized or diffuse motor impairment, and a precise description of clinical features is essential in order to allow a complete differential diagnosis. The two most common causes of acute flaccid paralysis in the pediatric emergency room are Guillain-Barré syndrome and transverse myelitis; notwithstanding, other etiologies should be considered, such as acute disseminated encephalomyelitis, infectious myelitis, myasthenia gravis, stroke, alternating hemiplegia of childhood, periodic paralyses, brainstem encephalitis, and functional muscle weakness. Algorithms for acute localized or diffuse weakness investigation in the emergency setting are also presented. Conclusions: The clinical skills to obtain a complete history and to perform a detailed physical examination are emphasized. An organized, logical, and stepwise diagnostic and therapeutic management is essential to eventually restore patient's well-being and full health.

Resumo Objetivos: Apresentar uma abordagem clínica, enfatizar a investigação diagnóstica, voltada para crianças e adolescentes no pronto-socorro com fraqueza muscular de surgimento agudo. Fontes: Foi feita uma pesquisa sistemática na base de dados PubMed entre abril e maio de 2017, com os seguintes termos de pesquisa em várias combinações: "agudo", "fraqueza", "déficit motor", "paralisia flácida", "criança", "pediátrico" e "emergência". Os trabalhos escolhidos para esta revisão foram publicados nos últimos dez anos, de 1997 a 2017. Este trabalho aborda a faixa etária pediátrica, até 18 anos. Resumo dos dados: O déficit motor agudo é uma causa razoavelmente comum para crianças e adolescentes procurarem o pronto-socorro. Os pacientes podem ser classificados como com deficiência motora localizada ou difusa e uma descrição precisa das características clínicas é essencial para possibilitar um diagnóstico diferenciado completo. As duas causas mais comuns de paralisia flácida aguda no pronto-socorro pediátrico são síndrome de Guillain-Barré e mielite transversa, independentemente de outras etiologias serem consideradas, como encefalomielite disseminada aguda, mielite infecciosa, miastenia grave, derrame, hemiplegia alternante da infância, paralisia periódica, encefalite do tronco encefálico e fraqueza muscular funcional. Os algoritmos da investigação de fraqueza aguda localizada ou difusa na configuração de emergência também são apresentados. Conclusões: São enfatizadas as habilidades clínicas para obter um histórico completo e fazer um exame físico detalhado. Um manejo diagnóstico e terapêutico organizado, lógico e por etapas é essencial para eventualmente restaurar o bem-estar e a saúde total do paciente.

Epstein-Barr Virus Infection associated Transverse Myelitis with Brain Involvement in an Immunosuppressed Patient: A Case Report

A 19-year-old girl with immunosuppressive agents of tacrolimus and mychophenolate mofetil following liver transplantation due to glycogen storage disease visited hospital due to lower extremity motor weakness and blurred vision. Motor power was checked as grade II in the upper extremities and grade 0 in the lower extremities with absence of deep tendon reflexes and anal sphincter dysfunction. The magnetic resonance imaging (MRI) showed increased T2 high signal intensity lesions from C4 to L2 level of spinal cord, cerebral cortex, and the left optic nerve. The cerebrospinal fluid (CSF) analysis showed pleocytosis. Epstein-Barr virus (EBV) deoxyribonucleic acid (DNA) was detected as 5,954 copies/mL in CSF whereas all other microbiologic tests were negative. Anti-aquaporin 4 antibody and oligoclonal band were not detected. Intravenous immunoglobulin, methylprednisolone pulse therapy and 3-week course of acyclovir were administered. Although motor power in the upper extremities recovered to grade V, motor power in the lower extremities did not show any improvement. The EBV viral load was not detected in the follow-up CSF examination. EBV infection in an immune-compromised patient could cause extensive demyelinating diseases in central nervous system and result in severe disability.

Acute Combined Central and Peripheral Demyelination in Children: in Comparison with Isolated Demyelinating Disease

PURPOSE: This study aimed to describe the clinical characteristics and outcomes of children with acute combined central and peripheral nervous system demyelination (CCPD); and compare with the children of isolated acute central or peripheral nervous system demyelination. METHODS: A retrospective chart review of 145 children with acute demyelinating disease between 2010 and 2015 was undertaken in children with younger than 18 years old. Among these, 96 fulfilled criteria (clinical features and positive neuroimaging or electromyography/nerve conduction studies) for either acute central (group A, n=60, 62.5%) or peripheral (group B, n=30, 31.3%) nervous system demyelination, or a CCPD (group C, n=6, 6.3%). RESULTS: Significant differences among the groups (A vs B vs C) were evident for occurrence of disease between 2013-2015 (45.0% vs 43.3% vs 83.3%; P=0.024), admission to intensive care unit (8.3% vs 26.7% vs 50.0%; P=0.027), length of hospitalization (median, 9.7 vs 12.3 vs 48.3 days; P<0.001), treatment with steroids (88.3% vs 10.0 vs 100.0%; P=0.003), immunoglobulins (13.3% vs 100.0% vs 100.0%; P=0.002) and plasmapheresis (0.0% vs 3.3% vs 50.0%; P=0.037) and severe disability at discharge (3.3% vs 16.7% vs 33.3%; P=0.012). Children of group C showed good response to simultaneous use of immunoglobulin and high-dose corticosteroids and earlier try of plasmapheresis, however, two patients had moderate degree of neurological disability. CONCLUSION: Systemic studies using neuroimaing and electromyography/nerve conduction studies in all patients with demyelinating disease will be necessary to verify the combined or isolated disease, because CCPD might have the poorer outcome than isolated disease.