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1.
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
2.
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
3.
Rinsho Ketsueki ; 59(12): 2588-2593, 2018.
Artigo em Japonês | MEDLINE | ID: mdl-30626794

RESUMO

Chronic graft versus host disease (cGVHD) of the central nervous system is a rare condition that could occur after allogeneic hematopoietic stem cell transplantation (SCT) but has been poorly documented. Here, we report a patient diagnosed with recurrent acute disseminated encephalomyelitis (ADEM) with longitudinal extensive transverse myelitis (LETM) as cGVHD after HLA haploidentical peripheral blood SCT using posttransplantation cyclophosphamide for mixed-phenotype acute leukemia. We observed clinical and radiological improvement after the rituximab treatment of the condition that had been refractory to steroids. To the best of our knowledge, no report of cGVHD presented recurrent ADEM with LETM after allogeneic SCT and successfully treated with rituximab. Hence, ADEM should be included in the differential diagnosis of neurological symptoms in posttransplant patients with cGVHD.


Assuntos
Encefalomielite Aguda Disseminada/tratamento farmacológico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Rituximab/uso terapêutico , Transplante Haploidêntico/efeitos adversos , Diagnóstico Diferencial , Doença Enxerto-Hospedeiro , Humanos , Medula Espinal/patologia
4.
J Neurol ; 264(8): 1754-1762, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28695361

RESUMO

Patients with acute disseminated encephalomyelitis (ADEM) are presumed to have radiological monophasic disease, but this is uncertain since follow-up brain MRI is not routinely performed. We aimed to ascertain combined radiological and clinical monophasic disease in ADEM patients and to assess whether performing early (<6 months) follow-up brain MRI has prognostic value for subsequent multiphasic disease. We retrospectively studied the medical records of patients initially diagnosed with ADEM (years 2000-2014) at the Massachusetts General Hospital, USA. A neuroimaging specialist, masked to clinical events, reviewed all available brain MRIs. We included 62 patients (25 male; 30 pediatric; median clinical follow-up 3 years) and classified them into two subgroups: (1) clinically monophasic (no new, recurrent or worsening neurological symptoms >3 months after onset) (n = 45), and (2) clinically multiphasic (clinical relapse >3 months after onset) (n = 17). All clinically monophasic patients with brain MRI follow-up (n = 30) also had radiological monophasic disease a median of 2 years after ADEM onset. New lesions (58 vs. 14%) and persistent lesions (100 vs. 18%) on early brain MRI [available in 40 patients (65%)], as well as clinical flares (53 vs. 20%), were more common in clinically multiphasic versus monophasic patients. These early follow-up data allowed us to predict multiphasic disease with reasonable accuracy in a multivariable model (AUC = 0.73). We conclude that performing early follow-up brain MRI routinely in ADEM patients would aid clinicians in predicting multiphasic disease and may stratify patients who would benefit from initiation of disease-modifying therapy for multiple sclerosis.


Assuntos
Encéfalo/diagnóstico por imagem , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Imagem por Ressonância Magnética , Adolescente , Adulto , Encefalomielite Aguda Disseminada/líquido cefalorraquidiano , Encefalomielite Aguda Disseminada/tratamento farmacológico , Feminino , Seguimentos , Humanos , Modelos Logísticos , Masculino , Análise Multivariada , Neuroimagem , Prognóstico , Estudos Retrospectivos , Sensibilidade e Especificidade , Medula Espinal/diagnóstico por imagem , Fatores de Tempo , Adulto Jovem
6.
BMJ Case Rep ; 20172017 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-28433979

RESUMO

A previously well 25-year-old man presented with agitation, double incontinence and left-sided incoordination. His symptoms started after smoking a synthetic cannabinoid (black mamba) 5 days earlier. Over 48 hours, he developed aphasia, generalised hypertonia, hyper-reflexia and dense left hemiparesis. This progressed to profuse diaphoresis, fever, tachycardia, hypertension and a possible seizure necessitating admission to the intensive care unit. CT head and cerebrospinal fluid analysis were unremarkable. MRI brain demonstrated asymmetric multifocal hyperintense lesions in white and grey matter, which raised suspicions of acute disseminated encephalomyelitis (ADEM). An electroencephalogram showed widespread brain wave slowing, indicating diffuse cerebral dysfunction. Cerebral angiogram was normal. Toxicology analysis of the substance confirmed a potent synthetic cannabinoid NM2201, technically legal at the time. The patient made a slow but significant recovery after a course of intravenous methylprednisolone, intravenous immunoglobulins and oral steroids, and was later transferred to a rehabilitation bed.


Assuntos
Canabinoides/toxicidade , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Substância Cinzenta/diagnóstico por imagem , Substância Branca/diagnóstico por imagem , Adulto , Encefalomielite Aguda Disseminada/induzido quimicamente , Encefalomielite Aguda Disseminada/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Imunoglobulinas Intravenosas/uso terapêutico , Imagem por Ressonância Magnética , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Esteroides/administração & dosagem , Esteroides/uso terapêutico , Resultado do Tratamento
7.
Rinsho Shinkeigaku ; 57(4): 174-179, 2017 04 28.
Artigo em Japonês | MEDLINE | ID: mdl-28367948

RESUMO

A 26-year-old, otherwise healthy man presented with visual abnormality followed by loss of consciousness and convulsion. The patient then developed headache and fever 14 days later. Brain MRI showed hyperintensities in the left cingulate cortex. The cerrebrospinal fluid examinations showed mononuclear pleocytosis and positive PCR results for human herpesvirus 6 (HHV-6). A diagnosis of HHV-6 encephalitis and symptomatic epilepsy was made. The patient's clinical symptoms improved promptly following acyclovir treatment. However, 3 months later the patient noticed dysesthesia in the trunk, the left upper limb and the right lower limb. Brain and spine MRI showed multiple brain white matter lesions, the middle cerebellar peduncle and cervical spinal lesions. The symptoms resolved following methylprednisolone pulse therapy only. We report an adult patient with HHV-6 encephalitis followed by acute disseminated encephalomyelitis whose initial presentation was epilepsy. HHV-6 encephalitis should be included in the differential diagnosis of encephalitis of unknown etiology in an immunocompetent adult.


Assuntos
Encefalite/diagnóstico , Encefalite/virologia , Encefalomielite Aguda Disseminada/etiologia , Herpesvirus Humano 6/isolamento & purificação , Imunocompetência , Infecções por Roseolovirus , Adulto , Biomarcadores/líquido cefalorraquidiano , Encéfalo/diagnóstico por imagem , Vértebras Cervicais , Encefalite/tratamento farmacológico , Encefalomielite Aguda Disseminada/tratamento farmacológico , Epilepsia/tratamento farmacológico , Epilepsia/etiologia , Humanos , Imagem por Ressonância Magnética , Masculino , Metilprednisolona/administração & dosagem , Pulsoterapia , Medula Espinal/diagnóstico por imagem , Resultado do Tratamento
8.
Eur J Paediatr Neurol ; 21(2): 358-366, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-27818023

RESUMO

BACKGROUND: Although the diagnosis of multiple sclerosis (MS) in the paediatric population remains challenging, paediatric-onset MS is increasingly recognized worldwide. METHODS: We report on the clinical and biochemical features of a Belgian multicentre cohort of paediatric MS patients in a national retrospective descriptive study. RESULTS: Twenty one paediatric MS patients from four Belgian University Hospitals were included. In nine patients, onset of MS was before the age of ten years which makes the study cohort of special interest. We report a higher incidence of acute disseminated encephalomyelitis (ADEM)-like first MS attacks and an overall higher proportion of polysymptomatic episodes than in adult and most paediatric cohorts reported in the literature. The clinical presentation in our cohort was rather severe with high median EDSS-score during the first clinical manifestation and barely more than half of our study patients showing full recovery after their first clinical manifestation. Also, a significant proportion of children in our cohort has severe disease progression despite disease modifying therapy and 9.5% of patients showed transition to secondary progressive multiple sclerosis during adolescence. CONCLUSION: An early and correct diagnosis of paediatric MS is essential to start early adequate treatment. As illustrated by our study cohort, current treatment options in childhood are unsatisfactory.


Assuntos
Encefalomielite Aguda Disseminada/epidemiologia , Esclerose Múltipla Crônica Progressiva/epidemiologia , Esclerose Múltipla/epidemiologia , Adolescente , Bélgica/epidemiologia , Criança , Pré-Escolar , Progressão da Doença , Encefalomielite Aguda Disseminada/complicações , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Feminino , Humanos , Incidência , Masculino , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico por imagem , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla Crônica Progressiva/complicações , Esclerose Múltipla Crônica Progressiva/diagnóstico por imagem , Esclerose Múltipla Crônica Progressiva/tratamento farmacológico , Neurite Óptica/complicações , Neurite Óptica/epidemiologia , Recidiva , Estudos Retrospectivos
9.
Conn Med ; 81(2): 103-106, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29738155

RESUMO

A 17-year-old female presentedwith acute disseminated encephalomyelitis (ADEM), a demyelinating disease ofthe central nervous system, fourweeks after receiving a meningococcalvaccination. The report highlights a rare but known presen- tation following routine vaccination that requires a high degree ofsuspicion in recognition and management. Early initiation of steroid therapy, followed by a steroid taper over one month resulted in a favorable outcome of complete neurological recovery.


Assuntos
Encefalomielite Aguda Disseminada/tratamento farmacológico , Encefalomielite Aguda Disseminada/etiologia , Glucocorticoides/uso terapêutico , Vacinas Meningocócicas/efeitos adversos , Vacinação/efeitos adversos , Adolescente , Encefalomielite Aguda Disseminada/diagnóstico , Feminino , Humanos , Fatores de Tempo , Resultado do Tratamento
10.
Mult Scler Relat Disord ; 10: 112-115, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27919476

RESUMO

Acute disseminated encephalomyelitis (ADEM) complicating dengue infection is still exceedingly rare even in endemic countries such as Malaysia. Here we report two such cases, the first in an elderly female patient and the second in a young man. Both presented with encephalopathy, brainstem involvement and worsening upper and lower limb weakness. Initial magnetic resonance imaging (MRI) of the brain was normal in the first case. Serum for dengue Ig M and NS-1 was positive in both cases. Cerebrospinal fluid (CSF) showed pleocytosis in both with Dengue IgM and NS-1 positive in the second case but not done in the first. MRI brain showed changes of perpendicular subcortical palisading white matter, callosal and brainstem disease mimicking multiple sclerosis (MS) in both patients though in the former case there was a lag between the onset of clinical symptoms and MRI changes which was only clarified on reimaging. The temporal evolution and duration of the clinical symptoms, CSF changes and neuroimaging were more suggestive of Dengue ADEM rather than an encephalitis though initially the first case began as dengue encephalitis. Furthermore in dengue encephalitis neuroimaging is usually normal or rarely edema, haemorrhage, brainstem, thalamic or focal lesions are seen. Therefore, early recognition of ADEM as a sequelae of dengue infection with neuroimaging mimicking MS and repeat imaging helped in identifying these two cases. Treatment with intravenous steroids followed by maintenance oral steroids produced good outcome in both patients.


Assuntos
Dengue/complicações , Dengue/diagnóstico por imagem , Encefalomielite Aguda Disseminada/complicações , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Esclerose Múltipla/diagnóstico por imagem , Adulto , Encéfalo/diagnóstico por imagem , Dengue/líquido cefalorraquidiano , Dengue/tratamento farmacológico , Diagnóstico Diferencial , Encefalomielite Aguda Disseminada/líquido cefalorraquidiano , Encefalomielite Aguda Disseminada/tratamento farmacológico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/líquido cefalorraquidiano
11.
BMC Neurol ; 16(1): 223, 2016 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-27855658

RESUMO

BACKGROUND: It is clinically rare to find cytomegalovirus (CMV)-associated encephalomyelitis in immunocompetent adults. Here, we present the case of an adult patient who developed acute transverse myelitis that was followed by immune-mediated disseminated encephalomyelitis. CASE PRESENTATION: A 38-year-old man developed acute paraplegia with paresthesia below the level of the T7-8 dermatome. Both brain and spinal cord MRIs performed at admission appeared normal. Corticosteroid therapy was initiated, with the later addition of high-dose intravenous immunoglobulins. After polymerase chain reaction analysis indicated the presence of CMV DNA in his cerebrospinal fluid (CSF), anti-viral therapy was added. Forty days after symptom onset, despite an initial positive response to this therapy, he developed dysarthria and truncal ataxia. Repeated magnetic resonance imaging scans demonstrated progressively expanding lesions involving not only the spinal cord but also the cerebral white matter, suggestive of extensive immune-mediated demyelination involving the central nervous system (CNS), as is observed in acute disseminated encephalomyelitis (ADEM). CONCLUSION: This case report underscores the importance of careful patient observation following the initial diagnosis of a CMV-associated CNS infection, such as transverse myelitis, on the possibility that post-infectious ADEM may appear.


Assuntos
Infecções por Citomegalovirus/diagnóstico , Citomegalovirus/isolamento & purificação , Encefalomielite Aguda Disseminada/diagnóstico , Imunoglobulinas Intravenosas/uso terapêutico , Mielite Transversa/diagnóstico , Adulto , Infecções por Citomegalovirus/líquido cefalorraquidiano , Infecções por Citomegalovirus/diagnóstico por imagem , Infecções por Citomegalovirus/tratamento farmacológico , Diagnóstico Diferencial , Encefalomielite Aguda Disseminada/líquido cefalorraquidiano , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Humanos , Hospedeiro Imunocomprometido , Imagem por Ressonância Magnética/métodos , Masculino , Mielite Transversa/líquido cefalorraquidiano , Mielite Transversa/diagnóstico por imagem , Mielite Transversa/tratamento farmacológico
12.
Rinsho Shinkeigaku ; 56(12): 846-851, 2016 12 28.
Artigo em Japonês | MEDLINE | ID: mdl-27840381

RESUMO

A 64-year-old man presented with acute onset of generalized seizure and encephalopathy. FLAIR images of brain MRI showed multifocal high-intensity lesions of the white matter. Within few days after the treatment with intravenous methylprednisolone (1,000 mg/day for 3 days), amelioration of clinical symptoms and recovery of MRI findings were observed. Six months after the withdrawal of oral steroid therapy, recurrent lesions were observed at the same locations as initially revealed on admission. Due to the concomitant development of peripheral lymphocytosis, a brain biopsy was performed from a right frontal lesion. Histological findings suggested extensive demyelination accompanied by infiltration of inflammatory lymphocytes and macrophages. In spite of the temporary remission after re-initiation of oral steroid therapy, reduction of oral steroid dosage resulted in new lesion formation apart from the initial locations. Based upon clinical features, MRI findings and histological examination, he was diagnosed with multiphasic disseminated encephalomyelitis (MDEM). Acute disseminated encephalomyelitis (ADEM) is one of common causes of demyelinating disease among children. However, multiphasic form of ADEM is particularly rare in adult patients. Here we reported a rare case of adult-onset MDEM, in which clinical, radiological and histological features were described, and efficacy of steroid therapy was highlighted.


Assuntos
Encefalomielite Aguda Disseminada/tratamento farmacológico , Metilprednisolona/administração & dosagem , Administração Oral , Azatioprina/administração & dosagem , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Doenças Desmielinizantes , Diagnóstico Diferencial , Quimioterapia Combinada , Edema , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/patologia , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Prednisolona/administração & dosagem , Pulsoterapia , Resultado do Tratamento
13.
Continuum (Minneap Minn) ; 22(5, Neuroimaging): 1613-1635, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27740991

RESUMO

PURPOSE OF REVIEW: This article focuses on neuroimaging in multiple sclerosis (MS), the most common central nervous system (CNS) demyelinating disorder encountered by practicing neurologists. Less common adult demyelinating disorders and incidental subclinical white matter abnormalities that are often considered in the differential diagnosis of MS are also reviewed. RECENT FINDINGS: Advancements in neuroimaging techniques, eg, the application of ultrahigh-field MRI, are rapidly expanding the use of neuroimaging in CNS demyelinating disorders. Probably the most important recent findings include the detection of cortical lesions and CNS atrophy even in early stages of MS. The key development for practicing neurologists is the growing impact of MRI on the diagnostic criteria for MS and neuromyelitis optica (NMO) spectrum disorders. SUMMARY: MRI serves as an important component of the diagnostic criteria for MS and other major CNS demyelinating disorders, and it has been established as a reliable and sensitive indicator of disease activity and progression. In addition, rapidly advancing neuroimaging techniques are helping to improve our understanding of disease pathogenesis.


Assuntos
Encefalomielite Aguda Disseminada/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Esclerose Múltipla Recidivante-Remitente/diagnóstico por imagem , Adolescente , Adulto , Doenças do Sistema Nervoso Central/diagnóstico por imagem , Doenças do Sistema Nervoso Central/tratamento farmacológico , Doenças Desmielinizantes/diagnóstico por imagem , Doenças Desmielinizantes/tratamento farmacológico , Encefalomielite Aguda Disseminada/tratamento farmacológico , Feminino , Humanos , Imunossupressores/administração & dosagem , Masculino , Esclerose Múltipla/diagnóstico por imagem , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/tratamento farmacológico , Esteroides/administração & dosagem
15.
Continuum (Minneap Minn) ; 22(3): 897-915, 2016 06.
Artigo em Inglês | MEDLINE | ID: mdl-27261688

RESUMO

PURPOSE OF REVIEW: This review summarizes a general approach to pediatric demyelination as well as specific features of each of the acquired demyelinating syndromes to help clinicians in their evaluation of children with these disorders. Case studies are included to illustrate the expanding phenotype of many of these syndromes. RECENT FINDINGS: With the creation of consensus definitions for the pediatric acquired demyelinating syndromes, recognition of demyelination in children has increased, as has understanding of the clinical and radiologic features, prognosis, and response to treatment. Collaborative studies and multicenter clinical trials are ongoing and needed to appropriately evaluate emerging therapies for some of the chronic demyelinating disorders, such as multiple sclerosis and neuromyelitis optica (NMO) spectrum disorder. SUMMARY: This review will aid the clinician in identifying key features of the pediatric acquired demyelinating syndromes and highlights a general approach for the diagnosis and treatment of these disorders.


Assuntos
Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Esclerose Múltipla/diagnóstico por imagem , Esclerose Múltipla/tratamento farmacológico , Mielite Transversa/diagnóstico por imagem , Mielite Transversa/tratamento farmacológico , Criança , Pré-Escolar , Doenças Desmielinizantes/diagnóstico por imagem , Doenças Desmielinizantes/tratamento farmacológico , Feminino , Humanos , Masculino , Metilprednisolona/administração & dosagem , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/tratamento farmacológico
16.
Crit Care Nurse ; 36(3): e1-6, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27252106

RESUMO

Acute disseminated encephalomyelitis is an inflammatory demyelinating disease of the central nervous system that has been associated with influenza immunization, but only a few cases related to vaccination for influenza have been reported. Acute disseminated encephalomyelitis developed in a 42-year-old woman within 3 weeks of receiving the seasonal influenza vaccine. She had 80% recovery after 3 months of treatment with methylprednisolone. Although cases of acute disseminated encephalomyelitis after vaccination for influenza are rare, enough of them have occurred that critical care nurses should be aware of the possibility. Early treatment can prevent serious residual signs and symptoms; therefore, correct and quick diagnosis is important. Medical history obtained from patients with central nervous system problems should include history of recent vaccinations.


Assuntos
Encefalomielite Aguda Disseminada/induzido quimicamente , Encefalomielite Aguda Disseminada/tratamento farmacológico , Vacinas contra Influenza/efeitos adversos , Metilprednisolona/administração & dosagem , Administração Oral , Adulto , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Vacinas contra Influenza/administração & dosagem , Infusões Intravenosas , Imagem por Ressonância Magnética/métodos , Pulsoterapia/métodos , Doenças Raras , Medição de Risco , Índice de Gravidade de Doença , Resultado do Tratamento
17.
BMJ Case Rep ; 20162016 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-27268491

RESUMO

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease that may occur in a postvaccination condition or as a parainfectious encephalomyelitis. It is almost always monophasic. The underlying pathogenesis of ADEM may include perivascular inflammation, oedema and demyelination in the central nervous system. We present a case of a 15-year-old girl who was diagnosed as having ADEM, as well as detected to be a follow-up case of autoimmune haemolytic anaemia on steroid treatment. She presented with progressive weakness of the right lower limb for the past 4 days. MRI showed multiple subcortical lesions of varying size showing hyperintensities in T2 fluid-attenuated inversion recovery (FLAIR). The patient responded well to steroid therapy. No residual lesion was found on follow-up. Very few cases have been found with this rare association in the literature.


Assuntos
Anemia Hemolítica Autoimune/complicações , Encefalomielite Aguda Disseminada/complicações , Adolescente , Anemia Hemolítica Autoimune/tratamento farmacológico , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Feminino , Humanos , Prednisolona/administração & dosagem , Esteroides/administração & dosagem
18.
Pediatrics ; 137(5)2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-27244783

RESUMO

Myelin oligodendrocyte glycoprotein (MOG) is a candidate target antigen in demyelinating central nervous system diseases, including acute disseminated encephalomyelitis (ADEM), neuromyelitis optica, and multiple sclerosis. It may give prognostic information regarding monophasic or recurrent course of the disease. MOG antibodies have been shown to be positive in high titers during the first episode of ADEM with rapidly decreasing to undetectable limits after recovery. However, persistent MOG antibodies are considered as a predicting factor for multiple sclerosis, optic neuritis relapses, and incomplete recovery of ADEM. Here we report a unique case with persistent MOG antibodies presented with multiphasic ADEM-like attacks. A 6-year-old girl was consulted with encephalopathy, gait disturbance, and oculomotor nerve palsy. Periventricular white matter lesions were seen on cranial magnetic resonance imaging studies. ADEM was diagnosed and treated with steroid. During follow-up, she experienced repeated episodes after steroid therapy termination. We were able to search MOG antibody at the ninth attack. The positivity of this antibody remained. It was thought to be associated with steroid-dependent course, and azathioprine and intravenous human immunoglobulin treatment were added. Patients with persistent MOG antibodies may benefit from addition of immunosuppressant agents, which may decrease the number of attacks.


Assuntos
Anticorpos/sangue , Encefalomielite Aguda Disseminada/imunologia , Imunossupressores/uso terapêutico , Glicoproteína Mielina-Oligodendrócito/imunologia , Substância Branca/patologia , Encéfalo/diagnóstico por imagem , Criança , Quimioterapia Combinada , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imagem por Ressonância Magnética , Metilprednisolona/uso terapêutico , Substância Branca/diagnóstico por imagem
19.
Neuropediatrics ; 47(4): 245-52, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27128728

RESUMO

Under the umbrella of pediatric-acquired demyelinating syndromes, there is a multitude of disorders, including optic neuritis, transverse myelitis, acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), and neuromyelitis optica spectrum disorders (NMOSD). Due to overlapping clinical and magnetic resonance imaging (MRI) features, it can be challenging to provide an accurate diagnosis. In view of therapeutic and prognostic implications, an early and reliable diagnosis is however of utmost importance. Recent studies of myelin oligodendrocyte glycoprotein (MOG) identify MOG, as a promising target for antibody-mediated demyelination and a biomarker for a relatively benign and non-MS disease course. We describe the clinical and MRI presentation of five children presenting with an acute, severe central nervous system inflammatory disease involving the brain and spinal cord, all of whom were positive for MOG-IgG antibody. Encephalopathy was uncommon at presentation and all had quick resolution of symptoms with intravenous steroid and intravenous immunoglobulin (IVIG) treatment. All patients recovered well, and have been treated with IVIG to potentially prevent relapses.


Assuntos
Autoanticorpos/imunologia , Encéfalo/diagnóstico por imagem , Doenças Desmielinizantes/fisiopatologia , Glicoproteína Mielina-Oligodendrócito/imunologia , Medula Espinal/diagnóstico por imagem , Criança , Pré-Escolar , Doenças Desmielinizantes/diagnóstico por imagem , Doenças Desmielinizantes/tratamento farmacológico , Doenças Desmielinizantes/imunologia , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/tratamento farmacológico , Encefalomielite Aguda Disseminada/imunologia , Encefalomielite Aguda Disseminada/fisiopatologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Imagem por Ressonância Magnética , Masculino , Metilprednisolona/uso terapêutico , Mielite Transversa/diagnóstico por imagem , Mielite Transversa/tratamento farmacológico , Mielite Transversa/imunologia , Mielite Transversa/fisiopatologia , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/tratamento farmacológico , Neuromielite Óptica/imunologia , Neuromielite Óptica/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento
20.
Intern Med ; 55(6): 699-702, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26984094

RESUMO

Acute disseminated encephalomyelitis (ADEM) followed by optic neuritis (ON) has been reported as a distinct phenotype associated with anti-myelin oligodendrocyte protein (MOG) antibody. We herein report the case of a 37-year-old woman who was diagnosed with ADEM at 4 years old of age and who subsequently developed ON followed by recurrent ADEM 33 years after the initial onset. A serum analysis showed anti-MOG antibody positivity. This phenotype has only previously been reported in pediatric cases. Neurologists thus need to be aware that the phenotype may occur in adult patients, in whom it may be assumed to be atypical multiple sclerosis.


Assuntos
Anti-Inflamatórios/uso terapêutico , Autoanticorpos/sangue , Encefalomielite Aguda Disseminada/diagnóstico , Glicoproteína Mielina-Oligodendrócito/imunologia , Neurite Óptica/fisiopatologia , Prednisolona/uso terapêutico , Adulto , Diagnóstico Diferencial , Encefalomielite Aguda Disseminada/tratamento farmacológico , Encefalomielite Aguda Disseminada/imunologia , Encefalomielite Aguda Disseminada/fisiopatologia , Feminino , Humanos , Imagem por Ressonância Magnética , Neurite Óptica/tratamento farmacológico , Neurite Óptica/imunologia , Resultado do Tratamento
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