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1.
Artigo em Inglês | MEDLINE | ID: mdl-36411077

RESUMO

BACKGROUND AND OBJECTIVES: Acute inflammatory CNS diseases include neuromyelitis optica spectrum disorders (NMOSDs) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Both MOGAD and acute disseminated encephalomyelitis (ADEM) have been reported after vaccination. Consequently, the mass SARS-CoV-2 vaccination program could result in increased rates of these conditions. We described the features of patients presenting with new acute CNS demyelination resembling NMOSDs or MOGAD within 8 weeks of SARS-CoV-2 vaccination. METHODS: The study included a prospective case series of patients referred to highly specialized NMOSD services in the UK from the introduction of SARS-CoV-2 vaccination program up to May 2022. Twenty-five patients presented with new optic neuritis (ON) and/or transverse myelitis (TM) ± other CNS inflammation within 8 weeks of vaccination with either AstraZeneca (ChAdOx1S) or Pfizer (BNT162b2) vaccines. Their clinical records and paraclinical investigations including MRI scans were reviewed. Serologic testing for antibodies to myelin oligodendrocyte glycoprotein (MOG) and aquaporin 4 (AQP4) was performed using live cell-based assays. Patients' outcomes were graded good, moderate, or poor based on the last clinical assessment. RESULTS: Of 25 patients identified (median age 38 years, 14 female), 12 (48%) had MOG antibodies (MOGIgG+), 2 (8%) had aquaporin 4 antibodies (AQP4IgG+), and 11 (44%) had neither. Twelve of 14 (86%) antibody-positive patients received the ChAdOx1S vaccine. MOGIgG+ patients presented most commonly with TM (10/12, 83%), frequently in combination with ADEM-like brain/brainstem lesions (6/12, 50%). Transverse myelitis was longitudinally extensive in 7 of the 10 patients. A peak in new MOGAD cases in Spring 2021 was attributable to postvaccine cases. Both AQP4IgG+ patients presented with brain lesions and TM. Four of 6 (67%) seronegative ChAdOx1S recipients experienced longitudinally extensive TM (LETM) compared with 1 of 5 (20%) of the BNT162b2 group, and facial nerve inflammation was reported only in ChAdOx1S recipients (2/5, 40%). Guillain-Barre syndrome was confirmed in 1 seronegative ChAdOx1S recipient and suspected in another. DISCUSSION: ChAdOx1S was associated with 12/14 antibody-positive cases, the majority MOGAD. MOGAD patients presented atypically, only 2 with isolated ON (1 after BNT162b2 vaccine) but with frequent ADEM-like brain lesions and LETM. Within the seronegative group, phenotypic differences were observed between ChAdOx1S and BNT162b2 recipients. These observations might support a causative role of the ChAdOx1S vaccine in inflammatory CNS disease and particularly MOGAD. Further study of this cohort could provide insights into vaccine-associated immunopathology.


Assuntos
COVID-19 , Encefalomielite Aguda Disseminada , Mielite Transversa , Neuromielite Óptica , Neurite Óptica , Feminino , Humanos , Glicoproteína Mielina-Oligodendrócito , Aquaporina 4 , Mielite Transversa/etiologia , Vacinas contra COVID-19/efeitos adversos , SARS-CoV-2 , Vacina BNT162 , COVID-19/prevenção & controle , Sistema Nervoso Central , Encefalomielite Aguda Disseminada/etiologia , Vacinação/efeitos adversos , Inflamação
2.
BMC Neurol ; 22(1): 418, 2022 Nov 09.
Artigo em Inglês | MEDLINE | ID: mdl-36352355

RESUMO

BACKGROUND: To explore the clinical characteristics and related factors of children with acute disseminated encephalomyelitis (ADEM) with positive anti-myelin oligodendrocyte glycoprotein (MOG) antibody. METHODS: A retrospective study was conducted and enrolled pediatric ADEM patients who underwent serum MOG antibody detection from May 2017 to August 2020. The patients were divided into two groups: MOG- immunoglobulin G (IgG) positive (n = 35) and MOG-IgG negative (n = 50). We analyzed the clinical characteristics of MOG-IgG-positive ADEM pediatric patients and conducted a comparative analysis between the two groups. RESULTS: Thirty-five patients (21 males and 14 females) in the MOG-IgG-positive group with encephalopathy, multifocal neurological symptoms, and typical magnetic resonance imaging (MRI) abnormalities were enrolled. They usually had a favorable outcome, while some suffered from relapse. Compared to the MOG-IgG-negative group, MOG-IgG-positive ADEM patients had a longer disease duration (median: 10 vs. 6 days), more meningeal involvement (31.4% vs. 8%) and frontal lobe involvement (82.8% vs. 68%), higher relapse rates (14.3% vs. 2%), lower serum tumor necrosis factor (1-12.4 pg/ml, median 1.7 vs. 1-34 pg/ml, median 2.2) and interferon-gamma (1-9.4 pg/ml, median 1.3 vs. 1-64 pg/ml, median 3) (P < 0.05, respectively). Multivariate logistic regression analysis showed that the longer disease duration, meningeal involvement and frontal lobe involvement were the correlated factors of patients with ADEM with MOG antibody (P < 0.05). CONCLUSIONS: Our findings provide clinical evidence that MOG-IgG positivity is associated with longer disease duration, meningeal involvement, and frontal lobe involvement.


Assuntos
Autoanticorpos , Encefalomielite Aguda Disseminada , Masculino , Feminino , Humanos , Glicoproteína Mielina-Oligodendrócito , Estudos Retrospectivos , Imunoglobulina G , Recidiva
3.
Biomed Res Int ; 2022: 6008375, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36425337

RESUMO

Acute hemorrhagic leukoencephalitis (AHLE), also called Hurst disease, is a rare demyelinating disease of the central nervous system (CNS) marked by rapid progression and acute inflammation of the white matter. Due to the correlation in their suspected postinfectious autoimmune pathogenesis, it is regarded as the most severe form of acute disseminated encephalomyelitis (ADEM). Because this clinical scenario has a high mortality rate, aggressive and immediate treatment is required. Although the exact cause of AHLE is unknown, it usually occurs after a bacterial or viral infection, or, less frequently, after a measles or rabies vaccination. AHLE has been reported in patients with coronavirus disease 2019 (COVID-19) as a rare but serious neurological complication. However, due to the lack of evidence-based diagnostic criteria, diagnosis is difficult. The small number of cases described in the literature, which most likely reflects underreporting and/or low incidence, necessitates greater public awareness. Increased clinical suspicion and early imaging identification of this entity may allow clinicians to pursue more aggressive treatment options, potentially reducing fatal outcomes. This study focuses on symptoms and causes of AHLE, difference between AHLE and ADME, diagnosis and treatment of AHLE, and its link with COVID-19.


Assuntos
COVID-19 , Encefalomielite Aguda Disseminada , Leucoencefalite Hemorrágica Aguda , Substância Branca , Humanos , Leucoencefalite Hemorrágica Aguda/diagnóstico , Leucoencefalite Hemorrágica Aguda/etiologia , Leucoencefalite Hemorrágica Aguda/terapia , COVID-19/complicações , COVID-19/diagnóstico , COVID-19/terapia , Encefalomielite Aguda Disseminada/complicações , Encefalomielite Aguda Disseminada/terapia , Substância Branca/patologia
4.
J Ayub Med Coll Abbottabad ; 34(3): 566-568, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36377177

RESUMO

We report a rare case of encephalitis that is not often described in clinical settings in neurology. Our case was 11-year-old female patient who had presented with features of meningoencephalitis, but not responded to the conventional treatment. Her magnetic resonance imaging revealed lesions in thalami, cerebellum and brainstem. The differentials in this age were infective and inflammatory causes of meningoencephalitis and acute disseminated encephalomyelitis (ADEM). Paraneoplastic was another differential. Mycoplasma serology came out positive. As a result, diagnosis of mycoplasma pneumoiae associated Rhombencephalitis was made based on diagnosis of exclusion.


Assuntos
Encefalomielite Aguda Disseminada , Meningoencefalite , Humanos , Feminino , Criança , Mycoplasma pneumoniae , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/etiologia , Imageamento por Ressonância Magnética
5.
Artigo em Inglês | MEDLINE | ID: mdl-36229191

RESUMO

BACKGROUND AND OBJECTIVE: The spectrum of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disorder (MOGAD) comprises monophasic diseases such as acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), and transverse myelitis and relapsing courses of these presentations. Persistently high MOG antibodies (MOG immunoglobulin G [IgG]) are found in patients with a relapsing disease course. Prognostic factors to determine the clinical course of children with a first MOGAD are still lacking. The objective of the study is to assess the clinical and laboratory prognostic parameters for a risk of relapse and the temporal dynamics of MOG-IgG titers in children with MOGAD in correlation with clinical presentation and disease course. METHODS: In this prospective multicenter hospital-based study, children with a first demyelinating attack and complete data set comprising clinical and radiologic findings, MOG-IgG titer at onset, and clinical and serologic follow-up data were included. Serum samples were analyzed by live cell-based assay, and a titer level of ≥1:160 was classified as MOG-IgG-positive. RESULTS: One hundred sixteen children (f:m = 57:59) with MOGAD were included and initially diagnosed with ADEM (n = 59), unilateral ON (n = 12), bilateral ON (n = 16), myelitis (n = 6), neuromyelitis optica spectrum disorder (n = 8) or encephalitis (n = 6). The median follow-up time was 3 years in monophasic and 5 years in relapsing patients. There was no significant association between disease course and MOG-IgG titers at onset, sex, age at presentation, or clinical phenotype. Seroconversion to MOG-IgG-negative within 2 years of the initial event showed a significant risk reduction for a relapsing disease course. Forty-two/one hundred sixteen patients (monophasic n = 26, relapsing n = 16) had serial MOG-IgG testing in years 1 and 2 after the initial event. In contrast to relapsing patients, monophasic patients showed a significant decrease of MOG-IgG titers during the first and second years, often with seroconversion to negative titers. During the follow-up, MOG-IgG titers were persistently higher in relapsing than in monophasic patients. Decrease in MOG-IgG of ≥3 dilution steps after the first and second years was shown to be associated with a decreased risk of relapses. In our cohort, no patient experienced a relapse after seroconversion to MOG-IgG-negative. DISCUSSION: In this study, patients with declining MOG-IgG titers, particularly those with seroconversion to MOG-IgG-negative, are shown to have a significantly reduced relapse risk.


Assuntos
Encefalomielite Aguda Disseminada , Neuromielite Óptica , Neurite Óptica , Humanos , Imunoglobulina G , Glicoproteína Mielina-Oligodendrócito , Recidiva Local de Neoplasia , Estudos Prospectivos , Síndrome
6.
Pharmazie ; 77(7): 262-269, 2022 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-36199182

RESUMO

Acute disseminated encephalomyelitis (ADEM) is a rare and immune-mediated inflammatory disorder of the central nervous system (CNS) that can be triggered by infections and vaccinations. To date, only anecdotal case studies have reported the association between ADEM incidence and seasonal influenza vaccines, and multiple studies have found no association. This study aimed to investigate the association between the incidence of ADEM and seasonal influenza vaccines in a real-world setting using data from the United States Vaccine Adverse Event Reporting System (VAERS). Further, propensity score matching and disproportionality analysis was performed by calculating the adjusted reporting odds ratio (ROR) of reported ADEM cases associated with seasonal influenza vaccines using multiple logistic regression. Additionally, we analysed the time-to-onset using Weibull shape parameters (WSPs). The VAERS database contained 390,352 adverse events reported from January 2011 to December 2020. The ROR of seasonal influenza vaccines for ADEM was 3.02 (95% confidence interval: 1.72-5.33). The median duration (interquartile range) of ADEM was 11.0 (5.0-33.0) days. The median duration of ADEM induced by egg culture-based influenza vaccine (Egg-based vaccine) and cell culture-based influenza vaccine (Cell-based vaccine) was 10.0 (5.0-24.0) and 91.0 (79.0-125.0) days (P < 0.001), respectively. Only Cell-based cases had WSP ß > 1, indicating a wear-out failure type. The incidence of ADEM within 30 days after administration of egg- and Cell-based vaccines was 78.6% and 0.0%, respectively. Our findings indicate that ADEM incidence is associated with seasonal influenza vaccines; thus, careful monitoring of ADEM is required within the first month of Egg-based vaccination and after two months of Cell-based vaccination. Neurologists and general practitioners should exercise caution, as the timing for careful monitoring varies depending on the vaccine type.


Assuntos
Encefalomielite Aguda Disseminada , Vacinas contra Influenza , Influenza Humana , Sistemas de Notificação de Reações Adversas a Medicamentos , Encefalomielite Aguda Disseminada/induzido quimicamente , Encefalomielite Aguda Disseminada/epidemiologia , Humanos , Vacinas contra Influenza/efeitos adversos , Influenza Humana/epidemiologia , Influenza Humana/prevenção & controle , Estações do Ano , Estados Unidos/epidemiologia , Vacinação/efeitos adversos
7.
Medicina (B Aires) ; 82 Suppl 3: 62-66, 2022 Aug 30.
Artigo em Espanhol | MEDLINE | ID: mdl-36054860

RESUMO

The pediatric neuroimmunology field has made significant progress in the last decade. Now, is possible to recognize primary demyelinating diseases, paraneoplastic syndromes, inflammatory (vasculitis), and granulomatous disorders that affect the central nervous system; at the same time, it is important to exclude neurologic manifestations caused by infections, toxic agents, and metabolic problems. An early diagnosis is imperative to institute treatment as soon as possible, improving outcomes. Treatment may include both, specific drugs if the etiology has been established, as well as drugs to treat potential complications, for example anticonvulsants, anti-inflammatory drugs, transfusions, or albumin replenishment within others. The main objective of this review is to provide guidance about the therapeutic options in pediatric autoimmune neurological diseases. We review the evidence and recommendations for the use of steroids in autoimmune demyelinating diseases, acute disseminated encephalomyelitis, optic neuritis, neuromyelitis optica, multiple sclerosis, among others. We will focus on current therapies, including high doses of intravenous methylprednisolone, followed by its progressive reduction, as well as intravenous immunoglobulin or plasmapheresis as second line therapies. Early institution of these treatments can save the patient's life and decrease their risk of permanent disability.


El campo de la pediatría neuro-inmunológica ha progresado significativamente en la última década. Ahora es posible reconocer con prontitud enfermedades desmielinizantes primarias, síndromes para-neoplásicos, enfermedades inflamatorias, autoinmunes y granulomatosas, que afectan el sistema nervioso central. Excluir con gran rapidez posibles causas infecciosas, agentes tóxicos, problemas metabólicos que se presenten con manifestaciones neurológicas es imperativo, ya que al hacer un diagnóstico preciso y temprano del paciente se puede instituir un tratamiento lo más pronto posible e incrementar las probabilidades de éxito. El tratamiento puede ser dirigido a la etiología específica, si se conoce. Adicionalmente, es importante tratar las complicaciones relacionadas a la propia enfermedad o efectos secundarios de los tratamientos que se impongan. El tratamiento puede incluir tanto fármacos específicos si se ha establecido la etiología, así como medicamentos para tratar posibles complicaciones, por ejemplo, anticonvulsivos, antiinflamatorios, transfusiones, o reposición de albúmina dentro de otros. El objetivo principal de esta revisión es brindar una guía sobre las opciones terapéuticas en enfermedades neurológicas autoinmunes en fase aguda. Revisamos la evidencia y recomendaciones acerca del uso de esteroides en enfermedades autoinmunes desmielinizantes, encefalomielitis aguda diseminada, neuritis óptica, neuromielitis óptica, esclerosis múltiple, entre otras, donde altas dosis de metilprednisolona, seguida por su disminución progresiva son esenciales, así como el uso de inmunoglobulina humana intravenosa y plasmaféresis, como tratamiento de segunda línea. La institución temprana de estos tratamientos puede salvar la vida del paciente y disminuir su discapacidad permanente.


Assuntos
Doenças Autoimunes , Encefalomielite Aguda Disseminada , Esclerose Múltipla , Neuromielite Óptica , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Criança , Encefalomielite Aguda Disseminada/tratamento farmacológico , Humanos , Metilprednisolona/uso terapêutico , Esclerose Múltipla/diagnóstico , Neuromielite Óptica/diagnóstico
8.
Ann Clin Transl Neurol ; 9(10): 1673-1678, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36053935

RESUMO

Several cases of autoimmune encephalitis have been reported after ChAdOx1 nCoV-19 (AZD1222) vaccination. We encountered a male patient who presented with generalized tonic-clonic seizures, cognitive decline, and gait disturbance that occurred suddenly after the second dose of the ChAdOx1 nCoV-19 vaccine. Clinical presentation and magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) test results were compatible with limbic encephalitis. Synaptic autoantibody tests confirmed serum and CSF GABA B receptor antibodies were present. The patient was treated with immunotherapy with intravenous immunoglobulin and rituximab. This GABA-B receptor antibody encephalitis case occurred presumably due to transient autoantibody production following vaccine administration.


Assuntos
COVID-19 , ChAdOx1 nCoV-19/efeitos adversos , Encefalite , COVID-19/prevenção & controle , Encefalite/induzido quimicamente , Encefalomielite Aguda Disseminada , Humanos , Imunoglobulinas Intravenosas , Masculino , Receptores de GABA-B , Rituximab , Vacinação/efeitos adversos
9.
JAAPA ; 35(9): 32-35, 2022 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-36007116

RESUMO

ABSTRACT: Acute disseminated encephalomyelitis (ADEM) is a relatively rare autoimmune process that causes demyelination of the central nervous system. This condition primarily affects children under age 10 years and can produce symptoms including fever, vomiting, headaches, and altered mental status. Diagnostic criteria include encephalopathy (behavioral changes or altered mental status not explained by fever) and MRI findings of demyelination during the first 3 months of developing symptoms without subsequent new MRI findings. Patients can have full recovery within days to weeks if recognized and treated promptly. This article describes an ED visit and hospital course for a 3-year-old girl with headaches and fatigue who was diagnosed with and treated for ADEM.


Assuntos
Encefalomielite Aguda Disseminada , Criança , Pré-Escolar , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/terapia , Feminino , Febre , Cefaleia , Humanos , Imageamento por Ressonância Magnética
10.
Brain Dev ; 44(10): 737-742, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36030148

RESUMO

BACKGROUND: Encephalitis due to vaccination for mumps is a rare complication that occurs in 0.00004% of recipients, and there has been no report of serious neurological sequelae. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) has been reported as the most frequent type among acute encephalopathy syndromes in the pediatric population in Japan. There has been no report of AESD caused by vaccinations. Case presentation We present the clinical course of a 1-year and 10-month-old boy who had no preexisting condition, and developed mumps vaccine-induced severe primary encephalitis. Refractory status epilepticus due to encephalitis persisted for 16 h and resulted in secondary encephalopathy as a form of AESD mimic. He had serious neurological sequelae, such as West syndrome, transient spastic tetraplegia, and intellectual disability, despite intensive treatments. DISCUSSION: The presented boy is the first patient to develop mumps vaccine-induced primary encephalitis with severe central nervous system (CNS) damage. Screening of the immunological background in the presented patient revealed no abnormalities; therefore, it is unclear why he developed such severe adverse events. In patients with acute encephalitis caused by the herpes simplex virus 1, inborn immune errors in CNS based on mutations in single genes are involved in its pathophysiology. Consequently, some immunogenetic alterations could be found by further analysis in the presented patient.


Assuntos
Encefalopatias , Encefalite Viral , Encefalite , Encefalomielite Aguda Disseminada , Caxumba , Estado Epiléptico , Masculino , Humanos , Criança , Lactente , Vacina contra Caxumba , Caxumba/complicações , Encefalopatias/etiologia , Encefalopatias/complicações , Convulsões/etiologia , Estado Epiléptico/etiologia , Estado Epiléptico/complicações , Encefalite/etiologia , Encefalite/complicações , Encefalomielite Aguda Disseminada/complicações , Febre/complicações
12.
Rev. neurol. (Ed. impr.) ; 75(2): 45-48, julio 2022. ilus
Artigo em Espanhol | IBECS | ID: ibc-207026

RESUMO

Introducción: COVID-19 (coronavirus disease-2019) es la enfermedad secundaria a la infección por el coronavirus de tipo 2 o SARS-CoV-2 (severe acute respiratory syndrome coronavirus type 2), que se ha constituido como pandemia desde diciembre de 2019. Si bien la afectación más frecuente y grave es la pulmonar, las complicaciones neurológicas secundarias a la COVID-19 son cada vez más reconocidas. La encefalomielitis aguda diseminada (EMAD) es una enfermedad autoinmune poco frecuente, clásicamente secundaria a una infección viral previa o concomitante. Existen informes de EMAD asociada a la COVID-19, casi todos con afectación respiratoria asociada. Presentamos el caso de una mujer joven diagnosticada con EMAD secundaria a la infección por el SARS-CoV-2 sin afectación respiratoria. Caso clínico: Mujer de 20 años que consultó por cuadro de desorientación y alteración conductual de una semana de evolución. Destaca en la historia la presencia de anosmia y sensación febril dos semanas antes del inicio de los síntomas neurológicos. En el examen físico destacó somnolencia, desorientación, hemianopsia homónima izquierda y síndrome piramidal ipsilateral. Se realizó una resonancia magnética encefálica que mostró múltiples lesiones inflamatorias desmielinizantes bihemisféricas de la sustancia blanca sugerentes de EMAD. La reacción en cadena de la polimerasa del SARS-CoV-2 en aspirado nasofaríngeo resultó positiva. Se descartaron otras causas de lesiones inflamatorias. Recibió esteroides con excelente respuesta. Conclusión: La EMAD es una complicación extremadamente rara en pacientes con COVID-19 que debe considerarse como una causa tratable de encefalopatía y/o déficits neurológicos multifocales en pacientes con infección activa o reciente por SARS-CoV-2 con o sin manifestaciones respiratorias.(AU)


Introduction: COVID-19, the disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), continues to grow all over the world since december of 2019. Although the main clinical manifestation is pulmonary disease, neurological manifestations are a prominent and increasingly recognized feature of the disease. The Acute Disseminated Encephalomyelitis (ADEM) is a rare autoimmune disorder, most commonly triggered by a viral infection. There are a few case reports of ADEM associated with COVID-19, almost all of them associated pulmonary disease. We report the case of a young patient with diagnosis of ADEM with SARS-CoV-2 infection without clinical respiratory symptoms. Case report: A 20-year-old woman with no relevant medical history was brought to the emergency department with a progressive confusional state lasted for 7 days. Family reported the development of smell and taste deficit since two weeks before the onset of neurological symptoms. There were no complaints of pulmonary symptoms. At admission, she was drowsy and disoriented. Left homonymous hemianopsia and an ipsilateral Babinski sign was identified. A brain magnetic resonance image was done showing multiple hyperintense bilateral, asymmetric patchy and poorly marginated lesions with gadolinium enhancement. She was SARS-CoV-2 PCR positive on nasopharyngeal swab. Intravenous high-dose glucocorticoids were administered with marked clinical improvement. Conclusion: ADEM is an extremely uncommon complication of SARS-CoV-2infection. Acute disseminated encephalomyelitis should be considered a potentially treatable cause of encephalopathy or multifocal neurological deficits in COVID-19 patients, even in the absence of respiratory symptoms.(AU)


Assuntos
Humanos , Feminino , Adulto Jovem , Encefalomielite Aguda Disseminada , Encefalite , Doenças Autoimunes , Substância Branca/patologia , Coronavirus , Imageamento por Ressonância Magnética
13.
Neurol India ; 70(3): 1244-1246, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35864679

RESUMO

Dengue is a common viral infection worldwide, though its neurological manifestations are infrequent (2%-11% in recent years) and can be varied as the Dengue virus per se is a non-neurotropic virus. Neurological manifestations of Dengue usually result from multisystem dysfunction which may be secondary to vascular leak or it can be due to direct virus invasion (dengue encephalitis) or an immunological phenomenon which is triggered by dengue infection (demyelinating disorders). Here we present two cases of dengue fever associated demyelinating disorders in two pediatric patients aptly depicting the two spectra of the disease. One of them is a case of fatal acute hemorrhagic leukoencephalopathy (AHLE) in a 3-year-old girl, that developed severe neurological sequelae while the other one is a case of an Acute disseminated Encephalomyelitis (ADEM) in a 3-year-old boy who had recovered with timely immunomodulatory therapy and appropriate management.


Assuntos
Dengue , Encefalite , Encefalomielite Aguda Disseminada , Criança , Pré-Escolar , Dengue/complicações , Dengue/diagnóstico , Encefalite/complicações , Encefalomielite Aguda Disseminada/complicações , Encefalomielite Aguda Disseminada/etiologia , Feminino , Humanos , Masculino
14.
Rev Neurol ; 75(2): 45-48, 2022 07 16.
Artigo em Espanhol | MEDLINE | ID: mdl-35822571

RESUMO

INTRODUCTION: COVID-19, the disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), continues to grow all over the world since december of 2019. Although the main clinical manifestation is pulmonary disease, neurological manifestations are a prominent and increasingly recognized feature of the disease. The Acute Disseminated Encephalomyelitis (ADEM) is a rare autoimmune disorder, most commonly triggered by a viral infection. There are a few case reports of ADEM associated with COVID-19, almost all of them associated pulmonary disease. We report the case of a young patient with diagnosis of ADEM with SARS-CoV-2 infection without clinical respiratory symptoms. CASE REPORT: A 20-year-old woman with no relevant medical history was brought to the emergency department with a progressive confusional state lasted for 7 days. Family reported the development of smell and taste deficit since two weeks before the onset of neurological symptoms. There were no complaints of pulmonary symptoms. At admission, she was drowsy and disoriented. Left homonymous hemianopsia and an ipsilateral Babinski sign was identified. A brain magnetic resonance image was done showing multiple hyperintense bilateral, asymmetric patchy and poorly marginated lesions with gadolinium enhancement. She was SARS-CoV-2 PCR positive on nasopharyngeal swab. Intravenous high-dose glucocorticoids were administered with marked clinical improvement. CONCLUSION: ADEM is an extremely uncommon complication of SARS-CoV-2infection. Acute disseminated encephalomyelitis should be considered a potentially treatable cause of encephalopathy or multifocal neurological deficits in COVID-19 patients, even in the absence of respiratory symptoms.


TITLE: Encefalomielitis aguda diseminada asociada a infección por el SARS-CoV-2 sin afectación respiratoria.Introducción. COVID-19 (coronavirus disease-2019) es la enfermedad secundaria a la infección por el coronavirus de tipo 2 o SARS-CoV-2 (severe acute respiratory syndrome coronavirus type 2), que se ha constituido como pandemia desde diciembre de 2019. Si bien la afectación más frecuente y grave es la pulmonar, las complicaciones neurológicas secundarias a la COVID-19 son cada vez más reconocidas. La encefalomielitis aguda diseminada (EMAD) es una enfermedad autoinmune poco frecuente, clásicamente secundaria a una infección viral previa o concomitante. Existen informes de EMAD asociada a la COVID-19, casi todos con afectación respiratoria asociada. Presentamos el caso de una mujer joven diagnosticada con EMAD secundaria a la infección por el SARS-CoV-2 sin afectación respiratoria. Caso clínico. Mujer de 20 años que consultó por cuadro de desorientación y alteración conductual de una semana de evolución. Destaca en la historia la presencia de anosmia y sensación febril dos semanas antes del inicio de los síntomas neurológicos. En el examen físico destacó somnolencia, desorientación, hemianopsia homónima izquierda y síndrome piramidal ipsilateral. Se realizó una resonancia magnética encefálica que mostró múltiples lesiones inflamatorias desmielinizantes bihemisféricas de la sustancia blanca sugerentes de EMAD. La reacción en cadena de la polimerasa del SARS-CoV-2 en aspirado nasofaríngeo resultó positiva. Se descartaron otras causas de lesiones inflamatorias. Recibió esteroides con excelente respuesta. Conclusión. La EMAD es una complicación extremadamente rara en pacientes con COVID-19 que debe considerarse como una causa tratable de encefalopatía y/o déficits neurológicos multifocales en pacientes con infección activa o reciente por SARS-CoV-2 con o sin manifestaciones respiratorias.


Assuntos
COVID-19 , Encefalomielite Aguda Disseminada , Adulto , COVID-19/complicações , Meios de Contraste , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/etiologia , Feminino , Gadolínio , Humanos , SARS-CoV-2 , Adulto Jovem
15.
Mult Scler Relat Disord ; 66: 104008, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35863128

RESUMO

BACKGROUND: Our aim was to propose criteria to distinguish multiple sclerosis (MS) from acute disseminated encephalomyelitis (ADEM) at onset based on age at onset, sex, cerebrospinl fluid (CSF)-specific oligoclonal bands, and MRI. METHODS: A neuroradiologist undertook retrospective evaluation of the baseline magnetic resonance imaging (MRI) in a nationwide cohort of children with medical record-validated MS (n = 67) and monophasic ADEM (n = 46). Children with ADEM had at least 5 years of follow-up for relapse. We used forward stepwise conditional logistic regression to develop our criteria based on age at onset, sex, CSF-specific oligoclonal bands, and MRI. We undertook sensitivity analyses using children with ADEM including encephalopathy and polyfocal neurological deficits and in those with onset between 11 and 17 years of age. We estimated accuracy statistics from our criteria and all previously proposed MRI criteria to distinguish MS and ADEM. RESULTS: The best performing criteria to differentiate MS from ADEM were scoring at least three points in the following categories: presence of CSF-specific oligoclonal bands (2 points), occipital lesion (1 point), age 11-17 years (1 point), female sex (1 point). These criteria gave highly reliable discrimination with sensitivity of 95% (95% CI=89%-100%), specificity of 100% (95% CI=100%-100%), and area under the curve of 98% (95% CI=95%-100%). The best performing MRI criteria had area under the curve of 84% (95% CI=78%-91%). Previously proposed MRI criteria had the following areas under the curve: Callen (75%), KIDMUS (82%), and McDonald 2017 criteria (68%). CONCLUSION: Combining sex, age at onset, CSF-specific oligoclonal bands, and MRI gives highly reliable differentiation between pediatric MS and monophasic ADEM at onset.


Assuntos
Encefalomielite Aguda Disseminada , Esclerose Múltipla , Adolescente , Criança , Estudos de Coortes , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Esclerose Múltipla/diagnóstico por imagem , Esclerose Múltipla/patologia , Bandas Oligoclonais , Estudos Retrospectivos
16.
Mult Scler Relat Disord ; 66: 104056, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35878513

RESUMO

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an immune-mediated encephalopathy with heterogeneous disease courses. However, clinical characteristics for a prognostication of functional recovery from acute episodes of ADEM remain limited. The study aims to characterize the clinical presentations and neuroimaging findings of children with poor functional recoveries from acute episodes of moderate to severe ADEM. METHODS: The multicenter retrospective cohort study included children under 18 years of age who presented with moderate to severe ADEM (modified Rankin Scale [mRS] ≥ 3 at nadir) from 2002 to 2019. Children were assigned to a good recovery group (mRS ≤ 2) and a poor recovery group (mRS ≥ 3) after mean 4.3 months of follow-up. The clinical presentations and the distribution of brain lesions on magnetic resonance imaging were compared between the two groups by the t-test for numerical variables and Fisher's exact test for categorical variables. Analyses of logistic regression were conducted and significant variables in the multivariate model were examined by the receiver operating characteristic curve for the prediction of functional recovery. RESULTS: Among the 73 children with moderate to severe ADEM, 56 (77%) had good functional recoveries and 17 (23%) showed poor functional recoveries. Children with poor recoveries had a lower rate of prodromal headache (12% vs. 39%, p = 0.04), and presented with higher proportions of dystonia (29% vs. 9%, p = 0.046), myoclonus (24% vs. 2%, p = 0.009), and cerebellar lesions on neuroimages (59% vs. 23%, p = 0.01). The multivariate analyses identified that a lack of prodromal headache (OR 0.1, 95% CI 0.005 - 0.7, p = 0.06) and the presentations of myoclonus (OR 21.6, 95% CI 1.7 - 874, p = 0.04) and cerebellar lesions (OR 4.8, 95% CI 1.3 - 19.9, p = 0.02) were associated with poor functional recoveries. These three factors could prognosticate poor outcomes in children with moderate to severe ADEM (area under the receiver operating characteristic curve 0.80, 95% CI 0.68 - 0.93, p = 0.0002). CONCLUSION: Nearly one-fourth of children with moderate to severe ADEM had a poor functional recovery from acute episodes, who were characterized by a lack of prodromal headache, the presentation of myoclonus, and the neuroimaging finding of cerebellar lesions. The clinical variables associated with poor functional recoveries could assist in the planning of immunotherapies during hospitalization for a better outcome in moderate to severe ADEM.


Assuntos
Encefalomielite Aguda Disseminada , Mioclonia , Adolescente , Criança , Encefalomielite Aguda Disseminada/complicações , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/terapia , Cefaleia/complicações , Humanos , Imageamento por Ressonância Magnética , Mioclonia/complicações , Prognóstico , Estudos Retrospectivos
17.
Mult Scler ; 28(13): 2112-2123, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-35822296

RESUMO

BACKGROUND: Limited information is available on associations between COVID-19 vaccines and central nervous system (CNS) demyelinating diseases. OBJECTIVES: We investigated potential safety signals for CNS demyelinating diseases related to COVID-19 vaccines using the World Health Organization pharmacovigilance database. METHODS: Disproportionality analyses of CNS demyelinating disease following COVID-19 vaccination were performed by calculating the information component (IC) or the reporting odds ratio (ROR) compared with those for the entire database and for all other viral vaccines. RESULTS: We identified 715 cases of optic neuritis, 515 of myelitis, 220 of acute disseminated encephalomyelitis (ADEM), and 2840 total CNS demyelinating events adverse drug reactions from July 2020 through February 2022. For mRNA-based and ChAdOx1 nCoV-19 vaccines, there were no potential safety signals of disproportionality for optic neuritis (IC025 = -0.93, ROR025 = 0.38; IC025 = -1.76, ROR025 = 0.26), myelitis (IC025 = -0.69, ROR025 = 0.50; IC025 = -0.63, ROR025 = 0.53), ADEM (IC025 = -1.05, ROR025 = 0.33; IC025 = -1.76, ROR025 = 0.20), or overall CNS demyelinating disease events (IC025 = -0.66, ROR025 = 0.52; IC025 = -1.31, ROR025 = 0.34) compared with other viral vaccines. CONCLUSION: As with other viral vaccines, our disproportionality analyses indicate that the risk of COVID-19 vaccine-associated CNS demyelinating disease was low.


Assuntos
COVID-19 , Encefalomielite Aguda Disseminada , Mielite , Neurite Óptica , COVID-19/epidemiologia , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Sistema Nervoso Central , ChAdOx1 nCoV-19 , Humanos , Mielite/etiologia , Neurite Óptica/etiologia , Farmacovigilância , RNA Mensageiro , Vacinação/efeitos adversos , Organização Mundial da Saúde
18.
Mult Scler Relat Disord ; 66: 104061, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35908447

RESUMO

Multiple sclerosis (MS) most commonly presents in young adults, although 3-5% of patients develop MS prior to the age of 18 years. The new and comprehensive consensus for the management of MS in Saudi Arabia includes recommendations for the management of MS and other CNS inflammatory demyelinating disorders in pediatric and adolescent patients. This article summarizes the key recommendations for the diagnosis and management of these disorders in young patients. Pediatric and adult populations with MS differ in their presentation and clinical course. Careful differential diagnosis is important to exclude alternative diagnoses such as acute disseminated encephalomyelitis (ADEM) or neuromyelitis optica spectrum disorders (NMOSD). The diagnosis of MS in a pediatric/adolescent patient is based on the 2017 McDonald diagnostic criteria, as in adults, once the possibility of ADEM or NMOSD has been ruled out. Few data are available from randomized trials to support the use of a specific disease-modifying therapy (DMT) in this population. Interferons and glatiramer acetate are preferred initial choices for DMTs based on observational evidence, with the requirement of a switch to a more effective DMT if breakthrough MS activity occurs.


Assuntos
Encefalomielite Aguda Disseminada , Esclerose Múltipla , Neuromielite Óptica , Adolescente , Criança , Humanos , Consenso , Acetato de Glatiramer/uso terapêutico , Interferons/uso terapêutico , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla/terapia , Neuromielite Óptica/epidemiologia , Arábia Saudita
19.
Front Immunol ; 13: 870867, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35757742

RESUMO

Background: Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disorder that is often misdiagnosed. To improve early diagnosis, we performed a systematic review and meta-analysis of clinical features, outcomes for ADEM in adults. Methods: The PubMed, Embase, Web of Science and Cochrane Library databases were searched for studies reporting the clinical features of adults with ADEM between January 1990 and May 2021. A random-effects meta-analysis model was used to pool data on clinical features and functional outcomes. Results: Twelve studies examining 437 adults with ADEM met the inclusion criteria. Overall, the clinical features and diagnostic findings observed in more than two-thirds of the patients were white matter lesions [87.1%, 95% confidence interval (CI)=75-95.6], polyfocal onset (80.5%, 95% CI=50.5-98.9) and pyramidal signs (68.7%, 95% CI =40.0-91.9). The mortality rate was 7.8% (95% CI = 3.3-13.5), and the risk of residual deficits was 47.5% (95% CI = 31.8-63.4). Conclusions: Adults with ADEM had worse outcomes than children. Clinicians should maintain high clinical suspicion for patients presenting with certain clinical features and diagnostic findings.


Assuntos
Encefalomielite Aguda Disseminada , Imageamento por Ressonância Magnética , Adulto , Criança , Progressão da Doença , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/patologia , Encefalomielite Aguda Disseminada/terapia , Humanos
20.
Am J Case Rep ; 23: e936574, 2022 Jun 19.
Artigo em Inglês | MEDLINE | ID: mdl-35717556

RESUMO

BACKGROUND Acute disseminated encephalomyelitis (ADEM) is a disorder of the central nervous system which has been associated with preceding infection as well as vaccinations. We present a case of a 61-year-old woman with ADEM after receiving her initial vaccination for the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This case highlights management of this acute condition. CASE REPORT A 61-year-old woman with history of hypertension and anxiety presented with progressive generalized weakness and difficulty with communication which began a few weeks ago, shortly after receiving the Pfizer vaccine for the novel coronavirus (COVID-19). On arrival, she was found to be encephalopathic and tachypneic, ultimately requiring emergent intubation. During her hospital course, an MRI of her brain was obtained which showed nonspecific acute versus subacute leukoencephalopathy involving the brainstem and deep white matter. Her cerebrospinal fluid showed elevated protein but was otherwise unremarkable. Further testing to rule out tick-borne illnesses, viral etiology, and multiple sclerosis were negative. Electroencephalography showed nonspecific diffuse cerebral dysfunction but no seizures or epileptiform discharges. She was treated with 5 doses of methylprednisolone 1 g and intravenous immunoglobulin (IVIG) 2 g/kg over 5 days. She had marked improvement in her neurologic status after treatment. CONCLUSIONS In conclusion, ADEM should be acknowledged as a rare but potential complication related to COVID-19 vaccination. A proper history and physical exam in addition to a thorough work-up are necessary for prompt recognition of this condition. Initial treatment should consist of steroids followed by IVIG versus plasmapheresis for those not responsive to steroids.


Assuntos
Vacinas contra COVID-19/efeitos adversos , COVID-19/prevenção & controle , Encefalomielite Aguda Disseminada/etiologia , SARS-CoV-2/imunologia , Vacinação/efeitos adversos , Encefalomielite Aguda Disseminada/complicações , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Leucoencefalopatias , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Fármacos Neuroprotetores/uso terapêutico , Taquipneia
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