Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 266
Filtrar
1.
BMJ Case Rep ; 13(12)2020 Dec 13.
Artigo em Inglês | MEDLINE | ID: mdl-33318286

RESUMO

A 53-year-old man admitted to the critical care secondary to respiratory failure due to COVID-19 developed agitation and global hypotonia. Brain MRI revealed bilateral hyperintense lesions throughout the brain and cerebrospinal fluid identified oligoclonal bands. Intravenous high-dose glucocorticoids were administered followed by an oral tapering dose and the patient clinically improved. Acute disseminated encephalomyelitis should be considered in patients with COVID-19 who present with altered mentation and polyfocal neurological deficits.


Assuntos
/complicações , Encefalomielite Aguda Disseminada/virologia , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuroimagem
2.
Neurol India ; 68(5): 1192-1195, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33109874

RESUMO

Objective: The purpose of this study was to report three patients COVID-19 infection with severe respiratory syndrome requiring intubation, who developed acute demyelinating encephalomyelitis (ADEM). Methods: Patient data were obtained from medical records from the North Memorial Hospital, Robbinsdale, MN, USA. Results: Three patients (two men and one woman, aged 38-63) presented with fatigue, cough, and fever leading to acute respiratory distress syndrome secondary to COVID-19 infection requiring ventilatory support. Two patients were unresponsive and the third patient had severe diffuse weakness. MRI in all patients showed findings consistent with ADEM. CSF showed elevated protein in all patients with normal cell count and no evidence of infection, including negative COVID-19 PCR. All three patients were treated with intravenous corticosteroids and one improved markedly. The other two had minimal response to steroids and no further improvement after IVIG. Conclusion: Neurological complications from COVID-19 are being rapidly recognized. Our three cases highlight the occurrence of ADEM as a postinfectious/immune-mediated complication of COVID-19 infection, which may be responsive to corticosteroid treatment.


Assuntos
Infecções por Coronavirus/complicações , Encefalomielite Aguda Disseminada/etiologia , Pneumonia Viral/complicações , Adulto , Idoso , Betacoronavirus , Transtornos da Consciência/etiologia , Diabetes Mellitus Tipo 2/complicações , Encefalomielite Aguda Disseminada/líquido cefalorraquidiano , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Hipertensão/complicações , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Obesidade/complicações , Pandemias , Gravidez , Complicações Infecciosas na Gravidez , Insuficiência Renal Crônica/complicações
3.
Rev. neurol. (Ed. impr.) ; 71(7): 257-269, 1 oct., 2020.
Artigo em Espanhol | IBECS | ID: ibc-195711

RESUMO

INTRODUCCIÓN: La enfermedad asociada a anticuerpos contra la glucoproteína del oligodendrocito asociado a la mielina (MOG) es una entidad infrecuente y prácticamente nueva en la medicina. En países en desarrollo, aún hay importantes limitaciones para la detección de los anticuerpos anti-MOG mediante ensayo basado en células, por lo que conocer las características clínicas de los diferentes fenotipos y sus diferencias con otras patologías desmielinizantes del sistema nervioso es fundamental, y con ello realizar un abordaje diagnóstico y terapéutico adecuado de los pacientes. OBJETIVO: Presentar una actualización en cuanto a las características clínicas del espectro de la enfermedad. Éste es el primer artículo en castellano que reúne los fenotipos más frecuentes y brinda una descripción clara de lo que se debe tener en cuenta en cada uno de ellos. DESARROLLO: Esta entidad se caracteriza por tener un curso monofásico o recurrente. La neuritis óptica es el fenotipo de presentación más frecuente en la población general, y la encefalomielitis aguda diseminada, la más frecuente en los niños. Otros fenotipos que se describen en la presente revisión son la mielitis transversa, la encefalitis cortical y los síndromes de tallo cerebral, así como los criterios propuestos para el diagnóstico de la enfermedad asociada a anticuerpos anti-MOG. CONCLUSIONES: En la actualidad no existen estudios que busquen caracterizar a la población hispanoparlante con esta enfermedad ni artículos de revisión en lengua castellana, por lo que es importante difundir conocimiento y desarrollar investigación en esta área


INTRODUCTION: Myelin oligodendrocyte glycoprotein (MOG) antibody disease is a rare and practically new entity in medicine. In developing countries, there are still important limitations for the detection of anti-MOG antibodies by cell-based assay, so knowing the clinical characteristics of the different phenotypes and their differences with other demyelinating pathologies of the central nervous system is essential in order to make a proper diagnostic and therapeutic approach of the patients. AIM: To present an update regarding the clinical characteristics of the disease spectrum, being the first article in Spanish that gathers the most frequent phenotypes and provides a clear description of what should be considered to identify each of these phenotypes. DEVELOPMENT: This disease is characterized by having a monophasic or recurrent course, with optic neuritis being the most frequent presentation phenotype in general population and disseminated acute encephalomyelitis the most frequent in children. Other phenotypes described in this review are transverse myelitis, focal cortical encephalitis and cerebral stem syndromes, as well as the proposed criteria for the diagnosis of the disease associated with MOG antibody disease. CONCLUSION: Currently there are no studies that seek to characterize the Spanish-speaking population with this disease, or review articles in Spanish, so it is important to disseminate knowledge and develop research in this area


Assuntos
Humanos , Glicoproteína Oligodendrócito-Mielina/análise , Encefalite/epidemiologia , Neurite Óptica/epidemiologia , Mielite Transversa/diagnóstico , Encefalite/diagnóstico , Glicoproteína Oligodendrócito-Mielina/imunologia , Mielite Transversa/imunologia , Encefalomielite Aguda Disseminada/tratamento farmacológico , Metilprednisolona/administração & dosagem
4.
J Neurol ; 267(10): 2799-2802, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32474657

RESUMO

A 51-year-old woman with COVID-19 infection developed coma and an impaired oculocephalic response to one side. MRI of the brain demonstrated acute multifocal demyelinating lesions, and CSF testing did not identify a direct cerebral infection. High-dose steroids followed by a course of IVIG was administered, and the patient regained consciousness over the course of several weeks. As more patients reach the weeks after initial infection with COVID-19, acute disseminated encephalomyelitis should be considered a potentially treatable cause of profound encephalopathy or multifocal neurological deficits.


Assuntos
Infecções por Coronavirus/complicações , Encefalomielite Aguda Disseminada/virologia , Pneumonia Viral/complicações , Anti-Inflamatórios/uso terapêutico , Betacoronavirus , Encefalomielite Aguda Disseminada/tratamento farmacológico , Encefalomielite Aguda Disseminada/patologia , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Pandemias
5.
Neurol India ; 68(1): 35-41, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32129240

RESUMO

Tumefactive demyelination is a phenomenon involving the radiographic resemblance of an acute demyelinating process in the central nervous system to neoplasia. Although this has been described and characterized for multiple sclerosis, it has been reported in a few cases in patients with acute disseminated encephalomyelitis (ADEM) within the past decade. While it may be challenging to establish a diagnosis of tumefactive ADEM according to clinical and radiological data alone, a thorough review of the clinical history and following the patient over time can be supportive of the same. The principal diagnostic confounds include neoplastic disease and a first attack of multiple sclerosis. A definitive diagnosis can be made by biopsy, which reveals perivenular demyelination and mononuclear cell infiltration in ADEM, in contrast to confluent plaque-like areas of demyelination in patients with multiple sclerosis. Histopathologic evidence of neoplastic disease includes characteristic features, including nuclear atypia and polymorphism, cellular hyperproliferation, mitoses, necrosis, endothelial proliferation, rosettes, and/or pseudorosettes. ADEM responds excellently to treatment with corticosteroids and is monophasic, with recurrence occurring infrequently. We review the literature on tumefactive ADEM and discuss the clinical manifestations, imaging characteristics, and histopathologic findings used to distinguish it from other conditions.


Assuntos
Encéfalo/patologia , Encefalomielite Aguda Disseminada/tratamento farmacológico , Esclerose Múltipla/tratamento farmacológico , Corticosteroides/uso terapêutico , Biópsia , Encefalomielite Aguda Disseminada/diagnóstico , Humanos , Imagem por Ressonância Magnética/métodos , Esclerose Múltipla/diagnóstico , Recidiva
6.
J Med Case Rep ; 13(1): 368, 2019 Dec 14.
Artigo em Inglês | MEDLINE | ID: mdl-31836009

RESUMO

BACKGROUND: Acute disseminated encephalomyelitis is generally preceded by an infection, and it is usually self-limiting and non-recurrent. However, when there are multiple attacks of acute disseminated encephalomyelitis followed by optic neuritis, it is defined as acute disseminated encephalomyelitis-optic neuritis. To the best of our knowledge, there are no previous reports of acute disseminated encephalomyelitis and optic neuritis preceded by autoinflammation, triggered by periodic fever syndrome. We report on a case of acute disseminated encephalomyelitis with optic neuritis and periodic fever syndrome in a 12-year-old Ecuadorian Hispanic boy with several relapses over the past 10 years, always preceded by autoinflammatory manifestations and without evidence of infectious processes. Whole exome sequencing was performed, and although the results were not conclusive, we found variants in genes associated with both autoinflammatory (NLRP12) and neurological (POLR3A) phenotypes that could be related to the disease pathogenesis having a polygenic rather than monogenic trait. CONCLUSION: We propose that an autoinflammatory basis should be pursued in patients diagnosed as having acute disseminated encephalomyelitis and no record of infections. Also, we show that our patient had a good response after 1 year of treatment with low doses of intravenous immunoglobulin and colchicine.


Assuntos
Encefalomielite Aguda Disseminada/fisiopatologia , Febre/imunologia , Imagem por Ressonância Magnética , Neurite Óptica/fisiopatologia , Administração Oral , Criança , Colchicina/uso terapêutico , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Encefalomielite Aguda Disseminada/imunologia , Febre/tratamento farmacológico , Supressores da Gota/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Neurite Óptica/diagnóstico por imagem , Neurite Óptica/tratamento farmacológico , Neurite Óptica/imunologia , Recidiva , Resultado do Tratamento , Sequenciamento Completo do Exoma
8.
Medicina (B Aires) ; 79 Suppl 3: 66-70, 2019.
Artigo em Espanhol | MEDLINE | ID: mdl-31603847

RESUMO

The two main demyelinating diseases in children are reviewed. Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS). For its physiopathological characteristics, probable etiologies, clinical manifestations, diagnosis, treatment, prognosis, evolution, as well as atypical alterations that complicate its diagnosis, the smaller the child is, more study is needed before reaching the diagnosis. The International Study Group of Multiple Pediatric Sclerosis, published the operating definitions for demyelinating diseases acquired from the central nervous system in children: the ADEM is monophasic, polysymptomatic and with encephalopathy. Its duration is up to 3 months, with fluctuating symptoms and magnetic resonance findings. MS is an isolated monofocal or polyfocal syndrome, without encephalopathy. Currently, two different and distinguishable diseases are considered from the onset of symptoms.


Assuntos
Encefalomielite Aguda Disseminada/diagnóstico por imagem , Esclerose Múltipla/diagnóstico por imagem , Encéfalo/diagnóstico por imagem , Encéfalo/fisiopatologia , Criança , Quimioterapia Combinada , Encefalomielite Aguda Disseminada/tratamento farmacológico , Humanos , Imunoterapia , Imagem por Ressonância Magnética , Esclerose Múltipla/tratamento farmacológico , Esteroides/uso terapêutico , Síndrome
9.
Acta Clin Croat ; 58(1): 173-179, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31363340

RESUMO

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that usually affects children and young adults. It most commonly has a monophasic course, although relapses are reported. Clinical presentation of the disease includes encephalopathy and multifocal neurological deficits. There are no established reliable criteria for diagnosis of ADEM and sometimes it is difficult to distinguish it from first attack of multiple sclerosis, especially in adults. The diagnosis of ADEM is based on clinical, radiological and laboratory findings. In the treatment of ADEM, high doses of corticosteroids, plasmapheresis and immunoglobulins are used. We report a case of a young adult female patient with ADEM who fully recovered after prompt administration of high dose methylprednisolone and immunoglobulins.


Assuntos
Corticosteroides/uso terapêutico , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Imunoglobulinas/uso terapêutico , Feminino , Humanos , Imagem por Ressonância Magnética , Metilprednisolona/uso terapêutico , Resultado do Tratamento , Adulto Jovem
10.
Pediatr Emerg Care ; 35(8): e150-e151, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31335784

RESUMO

Acute disseminated encephalomyelitis (ADEM) is exceptionally uncommon, with approximately 3 pediatric cases reported in the United States each year. Given the uncommon nature of ADEM, most of the current data rely heavily on case reports. The overwhelming majority of cases have been reported after an acute viral infection or vaccination. Although up to 90% of cases exhibit full remission after intravenous steroids, those in which treatment is delayed can display debilitating sequelae. Here, we present a case of ADEM in a 7-year-old boy who presented with double vision and imbalance with no recent history of acute viral infections or vaccinations.


Assuntos
Diplopia/etiologia , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Transtornos Neurológicos da Marcha/etiologia , Administração Intravenosa , Blefaroptose/etiologia , Criança , Diplopia/diagnóstico , Encefalomielite Aguda Disseminada/líquido cefalorraquidiano , Encefalomielite Aguda Disseminada/tratamento farmacológico , Transtornos Neurológicos da Marcha/diagnóstico , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Esteroides/administração & dosagem , Esteroides/uso terapêutico , Resultado do Tratamento
11.
Brain Behav ; 9(9): e01374, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31342665

RESUMO

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is an idiopathic inflammatory demyelinating disorder of the central nervous system (CNS). Early treatment is the key for neurological recovery. METHODS: A case of ADEM associated with varicella-zoster virus infection was presented, in which magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) examinations were included. RESULTS: Magnetic resonance imaging of the brain revealed multiple hyperintense lesions at the subcortical level on fluid-attenuated inversion recovery (FLAIR), and MRI of the spinal cord revealed longitudinally segmented hyperintense lesions at the spinal cord on T2-weighted images. The patient was treated with methylprednisolone and gancyclovir, and had a favorable recovery. Subsequent MRI of the brain and cervical cord showed the previous abnormal hyperintensities had markedly disappeared. CONCLUSION: A rare case of ADEM with longitudinal segmented hyperintense lesions at the spinal cord on T2-weighted images was presented. Excellent response to ADEM treatment with high-dose steroids was reported resulting in a remarkable neurological recovery. A long-term follow-up is needed for prognosis.


Assuntos
Encéfalo/diagnóstico por imagem , Encefalomielite Aguda Disseminada/complicações , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Medula Espinal/diagnóstico por imagem , Infecção pelo Vírus da Varicela-Zoster/complicações , Adolescente , Anti-Inflamatórios/uso terapêutico , Antivirais/uso terapêutico , Encéfalo/patologia , Encéfalo/virologia , Encefalomielite Aguda Disseminada/líquido cefalorraquidiano , Encefalomielite Aguda Disseminada/tratamento farmacológico , Feminino , Ganciclovir/uso terapêutico , Humanos , Metilprednisolona/uso terapêutico , Neuroimagem/métodos , Medula Espinal/virologia , Resultado do Tratamento , Infecção pelo Vírus da Varicela-Zoster/líquido cefalorraquidiano , Infecção pelo Vírus da Varicela-Zoster/diagnóstico , Infecção pelo Vírus da Varicela-Zoster/tratamento farmacológico
13.
Am J Emerg Med ; 37(11): 2004-2007, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-30846211

RESUMO

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an uncommon, treatable, primarily pediatric, immune-mediated disease. Diagnosis of ADEM requires two essential elements: typical clinical presentation and magnetic resonance imaging (MRI) findings. The aim of this study was to evaluate how clinical findings in the initial emergency department (ED) presentation influenced the timing of MRI. METHODS: A retrospective chart review was conducted of children diagnosed with ADEM, over a 12-year period, in a tertiary care pediatric center. Clinical presentation at ED admission was recorded and patients who underwent an MRI as part of their ED evaluation (early MRI) with those who had MRI performed during ward hospitalization (late MRI) were compared. RESULTS: 30 patients were diagnosed with ADEM during the study period. Encephalopathy and polyfocal neurological signs were described in 80% and 50% of patients ED charts, respectively. Seven patients underwent early MRI and polyfocal neurological signs were more common in this group (p = 0.006). Fever was more common in the late MRI group (p = 0.02). Following diagnosis, all patients were treated with immune-modulation therapy, improved clinically, and were discharged. CONCLUSION: 20% of ADEM patients were not encephalopathic at ED presentation. Polyfocal neurological signs and absence of fever at ED presentation were related to earlier MRI utilization and thus earlier diagnosis and treatment. Familiarity with the ADEM constellation of signs, and a high index of suspicion, may help the ED clinician in early diagnosis and treatment of this rare disease.


Assuntos
Encéfalo/diagnóstico por imagem , Encefalomielite Aguda Disseminada/diagnóstico , Imagem por Ressonância Magnética , Exame Neurológico , Adolescente , Criança , Pré-Escolar , Tomada de Decisões , Serviço Hospitalar de Emergência , Encefalomielite Aguda Disseminada/tratamento farmacológico , Encefalomielite Aguda Disseminada/fisiopatologia , Feminino , Febre/etiologia , Humanos , Imunoglobulinas/administração & dosagem , Lactente , Masculino , Prednisolona/administração & dosagem , Prednisolona/análogos & derivados , Estudos Retrospectivos , Convulsões/etiologia , Fatores de Tempo
15.
Acta Med Port ; 32(1): 81-85, 2019 Feb 01.
Artigo em Português | MEDLINE | ID: mdl-30753808

RESUMO

Acute disseminated encephalomyelitis is a rare inflammatory demyelinating multifocal disease of the central nervous system that typically occurs in children following vaccination or exanthematous viral infections and conveys an elevated risk of neurological sequelae unless promptly recognized and treated. We describe an adult case of acute disseminated encephalomyelitis following vaccination against Mumps, Measles and Rubella, presenting with fever and progressive neurological deficits which improved under systemic corticosteroid therapy. Considering the ongoing public debate regarding universal vaccination and the surge of previously controlled infectious diseases, we aim not only to underline the need for a rigorous assessment of vaccination safety on adult patients in order to prevent misguidance of public opinion, but also to alert clinicians for an early diagnosis of acute disseminated encephalomyelitis in these patients, the incidence of which we speculate may be rising.


Assuntos
Corticosteroides/uso terapêutico , Encefalomielite Aguda Disseminada/etiologia , Vacina contra Sarampo-Caxumba-Rubéola/efeitos adversos , Doença Aguda , Adulto , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Humanos , Masculino , Vacina contra Sarampo-Caxumba-Rubéola/administração & dosagem
16.
Mult Scler ; 25(1): 125-128, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30379117

RESUMO

Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are a well-recognized cause of acquired demyelinating syndromes in both adult and children. Despite basal ganglia involvement on imaging, movement disorder is not a cardinal feature. We describe a 2-year-9-month-old girl who presented with severe encephalopathy with aphasia, seizures and a complex movement disorder with dystonic posturing and tonic eye deviation. Neuroimaging revealed subtle asymmetrical predominantly white matter signal changes. MOG-Abs were positive in the serum. Other known pathogenic autoantibodies including N-methyl-D-aspartate receptor antibodies (NMDAR-Abs) were negative. The patient made a complete recovery following 2-week corticosteroid treatment. This case highlights the need for MOG-Ab testing in children with suspected autoimmune encephalopathies.


Assuntos
Encefalomielite Aguda Disseminada/diagnóstico , Transtornos dos Movimentos/diagnóstico , Glicoproteína Mielina-Oligodendrócito/imunologia , Corticosteroides/farmacologia , Pré-Escolar , Encefalomielite Aguda Disseminada/complicações , Encefalomielite Aguda Disseminada/tratamento farmacológico , Encefalomielite Aguda Disseminada/imunologia , Feminino , Humanos , Transtornos dos Movimentos/tratamento farmacológico , Transtornos dos Movimentos/etiologia , Transtornos dos Movimentos/imunologia
17.
Travel Med Infect Dis ; 28: 81-85, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-29574241

RESUMO

There are four neurological complications that can occur after malaria treatment at a time when the patient is aparasitaemic: delayed cerebellar ataxia, acute inflammatory demyelinating polyneuropathy, post-malaria neurological syndrome and acute disseminated encephalomyelitis (ADEM). The authors describe a case of a 54-year-old male who presented with encephalopathy and generalized seizures forty-three days after complete recovery from acute malaria by Plasmodium falciparum. Diagnosis of post-malaria ADEM was made based on the acute onset of the neurological symptoms, characteristic findings in magnetic resonance imaging of the brain and prompt response to steroid therapy. ADEM is an autoimmune demyelinating disease of the central nervous system that usually arises after an infection or vaccination. Its occurrence after malaria infection is relatively rare, and to the best of our knowledge there are only thirteen cases described in the literature.


Assuntos
Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/etiologia , Malária Falciparum/complicações , Doenças do Sistema Nervoso/etiologia , Encéfalo/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Humanos , Malária Falciparum/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/tratamento farmacológico , Esteroides/uso terapêutico , Resultado do Tratamento
18.
Curr Neurol Neurosci Rep ; 18(11): 76, 2018 09 18.
Artigo em Inglês | MEDLINE | ID: mdl-30229541

RESUMO

PURPOSE OF REVIEW: Diagnostic criteria for pediatric-onset multiple sclerosis (POMS) and related demyelinating disorders have been updated, neuroimaging studies have revealed new insights, biological assays identify patients with specific antibodies that influence both diagnosis and treatment, clinical trials are informing on treatment efficacy and safety, and longitudinal studies of neurological, cognitive and quality of life outcomes are informing on the impact of these diseases. We provide updates to assist providers caring for these children. RECENT FINDINGS: The recent 2017 McDonald Criteria for MS provide a simplified means to confirm diagnosis at onset and over time, and have been shown to be equally applicable for POMS. MRI analyses demonstrate that brain volume is reduced at onset, and that both volumetric and tissue integrity measures decline over time, indicating that POMS shares the degenerative aspects that also characterize adult-onset disease. The presence of myelin oligodendrocyte glycoprotein (MOG) antibodies at onset is detected in more than 50% of children with acute disseminated encephalomyelitis. When persistent over time, they are associated with relapsing disease. The first randomized clinical trials of disease supports superiority of fingolimod over subcutaneous interferon beta 1a, and demonstrated a favorable safety profile. Finally, while Expanded Disability Status Scale (EDSS) scores remain low in the first 10 years post-onset, POMS is associated with high rates of patient-reported fatigue and reduced engagement in exercise and carries a risk for cognitive impairment. The past 15 years have borne witness to a marked expansion in recognition and research in POMS. There are now more specific diagnostic criteria, antibodies to CNS proteins appear to define diagnostically distinct disorders, clinical trials have successfully launched and one has completed, and we are gaining increasing appreciation of the impact of MS and related disorders on the lived experience of children and adolescents.


Assuntos
Esclerose Múltipla/sangue , Esclerose Múltipla/diagnóstico por imagem , Adolescente , Adulto , Autoanticorpos/sangue , Criança , Diagnóstico Diferencial , Encefalomielite Aguda Disseminada/sangue , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Humanos , Imunossupressores/uso terapêutico , Estudos Longitudinais , Imagem por Ressonância Magnética/métodos , Esclerose Múltipla/tratamento farmacológico , Glicoproteína Mielina-Oligodendrócito/sangue , Neuroimagem/métodos , Qualidade de Vida , Ensaios Clínicos Controlados Aleatórios como Assunto/métodos , Resultado do Tratamento
19.
Medicina (B Aires) ; 78 Suppl 2: 75-81, 2018.
Artigo em Espanhol | MEDLINE | ID: mdl-30199370

RESUMO

Demyelinating diseases are a group of conditions of autoimmune etiology directed against the myelin of the central nervous system. In many cases, the onset of the illness is preceded by a nonspecific viral infection. Multiple sclerosis is a disease that evolves with relapses and remissions with polyfocal neurological deficits, being the most frequent optic neuritis, transverse myelitis and encephalic trunk involvement. Typically, magnetic resonance image (MRI) shows peri-ventricular, peri-callosal, cerebellum, brain stem and spinal cord hyperintensive lesions in T2 and FLAIR weighted images. Optic neuromyelitis is characterized by the presence of optic neuritis and transverse myelitis associated with the postrema and diencephalic area syndrome. MRI lesions are distributed in sectors rich with aquaporine-4 channels (AQP-4): hypothalamus, third and fourth ventricle, optic nerves and spinal cord. Finding anti AQP4 antibodies is useful for the diagnosis although they are not essential for it. Acute disseminated encephalomyelitis is typically a monophasic condition characterized by acute encephalopathy associated with hyperintense MRI large, bilateral and irregular asymmetric lesion in T2 and FLAIR weighted images. In all three cases, cerebral spine fluid (CSF) can show pleocytosis and hyperproteinorrachia. The presence of oligoclonal bands in CSF is characteristic of multiple sclerosis. In all cases, acute treatment includes high dose intravenous corticosteroids and plasmapheresis in non-responsive cases. Both multiple sclerosis and optic neuromyelitis require long-term treatment to prevent relapse and recurrent diseases.


Assuntos
Encefalomielite Aguda Disseminada/diagnóstico , Esclerose Múltipla/diagnóstico , Neuromielite Óptica/diagnóstico , Adolescente , Aquaporina 4 , Criança , Pré-Escolar , Meios de Contraste , Encefalomielite Aguda Disseminada/líquido cefalorraquidiano , Encefalomielite Aguda Disseminada/tratamento farmacológico , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Esclerose Múltipla/líquido cefalorraquidiano , Esclerose Múltipla/tratamento farmacológico , Neuromielite Óptica/líquido cefalorraquidiano , Neuromielite Óptica/tratamento farmacológico
20.
Eur J Neurol ; 25(5): 782-786, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29443442

RESUMO

BACKGROUND AND PURPOSE: Acute disseminated encephalomyelitis followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome that is different from multiple sclerosis and neuromyelitis optica spectrum disorder. The aim of this study was to describe the disease course, treatment response and outcome of children with ADEM-ON. METHODS: Children of <18 years of age were identified from six countries of the EU Paediatric Demyelinating Disease Consortium. Patients fulfilled the diagnostic criteria for ADEM followed by at least one ON. Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies were tested in all patients. RESULTS: In this study of 17 patients (nine boys) with ADEM-ON, anti-myelin oligodendrocyte glycoprotein (MOG) antibodies were identified in 16 patients. Age at onset was 6.1 years (interquartile range, 5.1-9.2 years). Twelve patients received oral prednisolone and 10 received maintenance immunosuppression (e.g. azathioprine, intravenous immunoglobulins, Rituximab). During a follow-up of 5.3 years (interquartile range, 1.8-10.2 years), 54 relapses occurred with a median of 3 relapses per patient (range, 1-9 per patient). Patients relapsed on all treatments but no relapses occurred on a prednisolone dose >10 mg/day. Visual and cognitive residual deficits were common in this group. CONCLUSIONS: Acute disseminated encephalomyelitis followed by optic neuritis is an anti-MOG antibody-associated relapsing disorder that can have a heterogeneous disease course. Patients were refractory for maintenance immunosuppression and appeared to be corticosteroid-dependent. Further international collaborations are now required to unify guidelines in this difficult-to-manage group of patients.


Assuntos
Encefalomielite Aguda Disseminada/diagnóstico , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Neurite Óptica/diagnóstico , Adolescente , Autoanticorpos , Azatioprina/uso terapêutico , Criança , Pré-Escolar , Progressão da Doença , Encefalomielite Aguda Disseminada/tratamento farmacológico , Encefalomielite Aguda Disseminada/imunologia , Feminino , Humanos , Masculino , Glicoproteína Mielina-Oligodendrócito/imunologia , Neurite Óptica/tratamento farmacológico , Neurite Óptica/imunologia , Prednisolona/uso terapêutico , Rituximab/uso terapêutico , Resultado do Tratamento
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA