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1.
BMJ Case Rep ; 17(4)2024 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-38589237

RESUMO

We reported a case of a school-going child, diagnosed with acute disseminated encephalomyelitis (ADEM) who presented with symptoms such as high fever, acute hemiplegia and ataxia and was referred for physiotherapeutic intervention. This case report aims to document the assessment and management of ADEM from the intensive care unit to the home setting by physical therapy. Also, the child developed ventilator-associated pneumonia and a right lower motor neuron facial injury for which the child was referred to paediatric physical therapy. Since then, continuing for 8 months has helped the child to be independent in all aspects of mobility with no complaints. The child showed improvement in WeeFIM scores and Sunnybrook facial grading after 99 sessions of intensive physical therapy for approximately 83 hours along with the home programme. It has been proven an efficient treatment method along with other medical lines of treatment for neurological impairment associated with ADEM.


Assuntos
Encefalomielite Aguda Disseminada , Modalidades de Fisioterapia , Criança , Humanos , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/terapia
2.
Mult Scler Relat Disord ; 84: 105497, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38364768

RESUMO

BACKGROUND: Prognostic markers for relapse and neurological disability following the first clinical event in children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) remain lacking. We investigated the clinical profiles and early prognostic factors associated with relapsing disease or impaired functional outcome in a large single-center cohort of pediatric MOGAD. METHODS: We retrospectively analyzed the clinical and paraclinical data and treatment outcomes of children with MOGAD seen at Children's Health in Dallas, Texas from 2009 to 2022. Univariate analyses were used to evaluate factors from initial event associated with relapsing disease course and impaired functional outcome (modified Rankin scale [mRS] >1) at final follow-up. RESULTS: Our cohort comprised of 87 children of diverse race/ethnicity. Presentation with acute disseminated encephalomyelitis (ADEM) was more frequent in children aged ≤8 years and Caucasian background, whereas presentation with optic neuritis was more common in children aged >8 years and other race/ethnicity. 44.3 % (27/61) had relapsing disease course, of whom 48.0 % had multiple relapses. 30.3 % (23/76) had final mRS >1. Children with abnormal electroencephalogram had reduced relapse risk. Children with ADEM presentation, severe disease, low MOG-IgG titer, and central and systemic inflammation (represented by cerebrospinal fluid pleocytosis and serum leukocytosis, respectively) at onset had higher likelihood of final mRS >1. CONCLUSION: Abnormal electroencephalogram at the first event was associated with reduced relapse risk while disease severity and peripheral inflammation significantly contributed to final neurological disability. Further studies are needed to validate these findings as early risk factors for disability and relapse and to identify optimal treatment strategies.


Assuntos
Autoanticorpos , Encefalomielite Aguda Disseminada , Criança , Humanos , Glicoproteína Mielina-Oligodendrócito , Estudos Retrospectivos , Encefalomielite Aguda Disseminada/diagnóstico , Inflamação , Doença Crônica , Progressão da Doença , Recidiva
3.
BMC Pediatr ; 24(1): 92, 2024 Feb 02.
Artigo em Inglês | MEDLINE | ID: mdl-38308211

RESUMO

BACKGROUND: The application of evoked potentials (EPs) to the diagnosis of acute disseminated encephalomyelitis (ADEM ) has not been investigated in detail. The aim of this study, therefore, was to analyze the value of multimodal EPs in the early diagnosis of pediatric ADEM. METHODS: This was a retrospective study in which we enrolled pediatric ADEM patients and controls (Cs) from neurology units between 2017 and 2021. We measured indices in patients using brainstem auditory evoked potentials (BAEPs), visual evoked potentials (VEPs) and somatosensory evoked potentials (SEPs), and then we analyzed their early diagnostic value in ADEM patients. RESULTS: The mean age of the ADEM group was 6.15 ± 3.28 years (range,1-12 years) and the male/female ratio was 2.1:1 The mean age of the Cs was 5.97 ± 3.40 years (range,1-12 years) and the male/female ratio was 1.3:1. As we used magnetic resonance imaging (MRI) as the diagnostic criterion, the sensitivity, specificity, and accuracy (κ was 0.88) of multimodal EPs were highly consistent with those of MRI; and the validity could be ranked in the following order with respect to the diagnosis of ADEM: multimodal Eps > single SEP > single VEP > single BAEP. Of 34 patients with ADEM, abnormalities in multimodal EPs were 94.12%, while abnormalities in single VEPs, BAEPs and SEPs were 70.59%,64.71%and 85.3%, respectively. We noted significant differences between single VEP/BAEPs and multimodal EPs (χ2 = 6.476/8.995,P = 0.011/0.003). CONCLUSIONS: The combined application of multimodal EPs was superior to BAEPs, VEPs, or SEPs alone in detecting the existence of central nerve demyelination, and we hypothesize that these modalities will be applicable in the early diagnosis of ADEM.


Assuntos
Encefalomielite Aguda Disseminada , Potenciais Evocados Visuais , Humanos , Criança , Feminino , Masculino , Lactente , Pré-Escolar , Encefalomielite Aguda Disseminada/diagnóstico , Estudos Retrospectivos , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Potenciais Somatossensoriais Evocados/fisiologia
4.
Medicina (Kaunas) ; 60(1)2024 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-38276067

RESUMO

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder that typically follows an infection or recent vaccination. Symptoms such as encephalopathy and focal neurological deficits appear weeks after the initial illness, leading to swift and progressive neurological decline. While ADEM in the brain has been well documented, reports of ADEM, specifically in the spinal cord, are relatively limited. A 58-year-old male presented with rapidly progressive bilateral lower extremity tingling, numbness, and mild gait disturbance approximately two days prior to visiting the emergency room. Spinal magnetic resonance imaging revealed a diffuse, longitudinal, high-signal lesion with mild enlargement of the conus and proximal cauda equina. The lesions were predominantly localized in the distal conus and cauda equina, and serial electrodiagnostic studies showed that the lesions progressed toward the proximal conus in tandem with symptom evolution and lacked clear lateralization. The patient was subsequently treated with high-dose steroids for seven days (intravenous methylprednisolone, 1 mg/kg). The patient's lower extremity weakness gradually improved and he was able to walk independently under supervision three weeks after symptom onset. In this case of spinal ADEM in a middle-aged adult, high-dose steroid treatment led to outstanding neurological recovery from both the initial occurrence and subsequent attacks.


Assuntos
Encefalomielite Aguda Disseminada , Compressão da Medula Espinal , Masculino , Pessoa de Meia-Idade , Adulto , Humanos , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/tratamento farmacológico , Compressão da Medula Espinal/complicações , Compressão da Medula Espinal/patologia , Encéfalo/patologia , Imageamento por Ressonância Magnética/efeitos adversos , Dano Encefálico Crônico
5.
Ther Apher Dial ; 28(1): 119-124, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37646345

RESUMO

INTRODUCTION: Acute disseminated encephalomyelitis is a rare acute demyelinating disease of the central nervous system (CNS). The pathogenesis remains unclear but is suspected to be autoimmune. High doses of methylprednisolone (HDMP) are currently considered standard of treatment. Plasmapheresis (PE) is typically given in steroid refractory cases. There is currently limited evidence supporting its use in ADEM. MATERIALS AND METHODS: We report a 16-year-old girl with ADEM who improved rapidly after initiating PE. RESULTS: The patient presented with acute onset of multifocal CNS symptoms, including encephalopathy, requiring intensive care unit management. Despite HDMP administration, her clinical condition continued to deteriorate. PE was therefore initiated on the same day as HDMP. Her clinical condition improved significantly following the first session. She was extubated and discharged from the intensive care unit the following day. CONCLUSION: HDMP combined with PE may be an effective first-line treatment in patients with fulminant ADEM.


Assuntos
Encefalomielite Aguda Disseminada , Mielite Transversa , Humanos , Feminino , Adolescente , Encefalomielite Aguda Disseminada/terapia , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/etiologia , Mielite Transversa/terapia , Mielite Transversa/complicações , Plasmaferese , Metilprednisolona/uso terapêutico , Unidades de Terapia Intensiva , Imageamento por Ressonância Magnética
6.
Rev Med Inst Mex Seguro Soc ; 61(5): 695-701, 2023 Sep 04.
Artigo em Espanhol | MEDLINE | ID: mdl-37773190

RESUMO

Background: Acute disseminated encephalomyelitis is an autoimmune and demyelinating disease. It is rare in adults. It has 3 main variants. One of them is Weston-Hurst syndrome, also called acute hemorrhagic leukoencephalitis. The objective was to share the experience in the diagnostic and therapeutic approach of this rare disease, as well as make a review of the current bibliography, in order to collaborate in the knowledge of this disease. Clinical case: 27-year-old woman, with a viral respiratory infection 2 weeks prior to the development of a neurological syndrome characterized by paresthesia, motor deficit, status epilepticus and acute encephalopathy, progressing rapidly to coma, with evidence in MRI of diffuse hemorrhagic lesions in cerebral white matter with demyelination and peripheral edema. It was administered steroid treatment for 5 days, with improvement of symptoms, but with motor and sensory deficits persisting. Conclusion: Acute disseminated encephalomyelitis and its variants are rare entities, with an important range of differential diagnosis, which must be identified and quickly treated to avoid their lethal or disabling outcome.


Introducción: la encefalomielitis aguda diseminada es una enfermedad autoinmune y desmielinizante. Es rara en el adulto. Cuenta con tres variantes principales. Una de ellas es el síndrome de Weston Hurst, también conocido como leucoencefalitis hemorrágica aguda. El objetivo fue compartir la experiencia en el abordaje diagnóstico y terapéutico de esta rara enfermedad, así como hacer una revisión de la bibliografía actual, a fin de colaborar con el conocimiento de esta. Caso clínico: mujer de 27 años con cuadro de infección respiratoria viral 2 semanas previas al desarrollo de síndrome neurológico caracterizado por parestesias, déficit motor, estatus epiléptico y encefalopatía aguda, el cual progresó a estado de coma y evidenció en resonancia magnética lesiones difusas hemorrágicas en sustancia blanca cerebral con desmielinización y edema periférico. Se inició tratamiento con esteroides por 5 días con mejora de síntomas, aunque persistió el déficit motor y sensitivo. Conclusión: la encefalomielitis aguda diseminada y la variante hemorrágica de esta son entidades raras, con una importante gama de diagnóstico diferencial, que deben ser identificadas y tratadas de forma rápida para evitar su letal o incapacitante desenlace.


Assuntos
Encefalomielite Aguda Disseminada , Leucoencefalite Hemorrágica Aguda , Estado Epiléptico , Adulto , Feminino , Humanos , Leucoencefalite Hemorrágica Aguda/diagnóstico , Leucoencefalite Hemorrágica Aguda/etiologia , Leucoencefalite Hemorrágica Aguda/patologia , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/etiologia , Encefalomielite Aguda Disseminada/patologia , Imageamento por Ressonância Magnética/efeitos adversos
8.
Tijdschr Psychiatr ; 65(6): 388-391, 2023.
Artigo em Holandês | MEDLINE | ID: mdl-37434580

RESUMO

We describe a case of a 36-year-old woman with no psychiatric or somatic history who was presented to the emergency department with a profound change in mental status, more precisely a catatonic status and auditory hallucinations. Due to the unclear aetiology and suspicion of underlying psychiatric problems, the patient was admitted to the psychiatric ward. After discharge against medical advice, readmission was necessary due to deterioration and sudden onset of myoclonus. On further examination, acute disseminated encephalomyelitis (ADEM) was diagnosed. This case illustrates that ADEM can present itself as an initial psychiatric problem and emphasizes the importance of extensive medical clearance at presentation and continued attention for possible somatic origin, even when the initial clearance turns out to be negative.


Assuntos
Catatonia , Encefalomielite Aguda Disseminada , Feminino , Humanos , Adulto , Encefalomielite Aguda Disseminada/diagnóstico , Serviço Hospitalar de Emergência , Hospitalização
9.
Rinsho Shinkeigaku ; 63(8): 518-522, 2023 Aug 29.
Artigo em Japonês | MEDLINE | ID: mdl-37518016

RESUMO

The patient is an 18-year-old female. She had a history of acute disseminated encephalomyelitis at the age of 6 and 7. She visited our hospital due to acute disturbance of consciousness, quadriplegia, and numbness of left upper and lower extremities. Brain MRI showed multiple DWI/FLAIR high-signal lesions in the bilateral cerebral hemispheres, cerebellum, and brainstem. Qualitative test indicated that serum anti-MOG antibodies was positive, and she was diagnosed with anti-MOG antibody-positive polyphasic disseminated encephalomyelitis. Intravenous mPSL pulse therapy was performed twice, but the symptoms worsened. As a second line treatment, plasma exchange was started. However, she developed transfusion related acute lung injury. Alternatively, she was treated with immunoadsorption plasmapheresis. Her symptoms were significantly improved. This case seems to be valuable because there are few reports showing effectiveness of immunoadsorption therapy on anti-MOG antibody-related diseases, especially for polyphasic disseminated encephalomyelitis.


Assuntos
Encefalomielite Aguda Disseminada , Feminino , Humanos , Autoanticorpos , Encefalomielite Aguda Disseminada/etiologia , Encefalomielite Aguda Disseminada/terapia , Encefalomielite Aguda Disseminada/diagnóstico , Glicoproteína Mielina-Oligodendrócito , Oligodendroglia , Plasmaferese/efeitos adversos
10.
Pediatr Neurol ; 146: 44-49, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37429226

RESUMO

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disorder of the central nervous system that is usually triggered by infections. We aimed to determine the temporal trends in the incidence of ADEM before and during the pandemic and their correlation with viral epidemiology. METHODS: We conducted a nationwide, population-based, retrospective, ADEM cohort study by using the Health Insurance Review and Assessment Service database. New-onset ADEM was defined as a patient (age <19 years) who was hospitalized with a diagnostic code of G04.0, G36.8, and G36.9 and a prescription of intravenous methylprednisolone. The National Infectious Disease Surveillance System was used to collect the nationwide viral epidemics. RESULTS: A total of 185 new-onset pediatric ADEM cases were included. The mean patient age was 7.0 ± 4.9 years. The incidence of ADEM was 0.34 to 0.48 of 100,000 persons per year before the pandemic, which dropped to 0.22 of 100,000 persons per year during the first pandemic year. The risk of ADEM occurrence was approximately 1.74% higher during the prepandemic years compared with the first pandemic year (odds ratio = 1.017, P = 0.009). There was a weak positive correlation between acute respiratory viral infection and ADEM incidence (r = 0.28, P = 0.03). CONCLUSION: This study demonstrates how infection control during the early coronavirus disease 2019 (COVID-19) pandemic influenced the incidence of ADEM. The low incidence of ADEM in the early COVID-19 pandemic may be related to the decline in acute respiratory viral infections, which are potential triggers of ADEM.


Assuntos
COVID-19 , Encefalomielite Aguda Disseminada , Criança , Humanos , Adulto Jovem , Adulto , Pré-Escolar , Encefalomielite Aguda Disseminada/diagnóstico , Pandemias , COVID-19/epidemiologia , COVID-19/complicações , Estudos de Coortes , Estudos Retrospectivos , Incidência
11.
Mult Scler Relat Disord ; 76: 104781, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37295322

RESUMO

Postinfectious neurological syndromes (PINS), among which acute disseminated encephalomyelitis (ADEM), are inflammatory and mostly monophasic disorders. We previously reported that PINS patients can show relapses, or even disease progression. Here we describe a cohort of patients with progressive-PINS and >5 years of follow-up, that developed a progressive worsening without radiological/cerebrospinal fluid analysis evidence of inflammation. At onset 5 patients fulfilled diagnostic criteria for ADEM and none for MS. Progression occurred after a median of 22 months from onset (in 4/7 after 1/more relapses), manifesting as ascending tetraparesis with bulbar functions involvement in 5/7. Five/7 patients received high dose steroids and/or IvIG and 6/7 Rituximab(n = 4) and/or cyclophosphamide(n = 2), with no impact on disease progression in 6/7. NfL levels were higher in patients with progressive-PINS compared to monophasic-ADEM (p = 0.023) and healthy controls (p = 0.004). Progression is rare, but possible, in PINS. Immunotherapy seems to be ineffective in these patients, and elevated serum NfL in serum suggest persistent axonal damage.


Assuntos
Encefalomielite Aguda Disseminada , Filamentos Intermediários , Humanos , Seguimentos , Encefalomielite Aguda Disseminada/diagnóstico , Progressão da Doença , Recidiva
12.
J Clin Neurosci ; 111: 57-70, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-36963124

RESUMO

BACKGROUND: Although global vaccination against COVID-19 infection has its excellence, potential side effects are yet of concern. Several lines of evidence have proposed ADEM occurrence after SARS-CoV-2 infection. Moreover, a large number of case reports and case series have also suggested the casual association between ADEM and COVID-19 vaccination. To better understand the development of ADEM following COVID-19 vaccination and its potential association, we aimed to systematically review ADEM cases reported after COVID-19 vaccination. METHODS: We conducted a comprehensive systematic search using three databases including PubMed, Scopus, and Web of Science. Studies that reported ADEM after COVID-19 vaccination were eligible to include in our study. Observational studies, case reports, and case series which reported cases of ADEM with sufficient detail to confirm clinical diagnosis following COVID-19 vaccination were eligible to enter our study. RESULTS: Twenty studies were included in our systematic review after the abstract and full-text screening with a total of 54 cases. Among included patients, 45 (85.1 %) developed ADEM after the first dose of the COVID-19 vaccine, and seven (12.9 %) cases experienced ADEM after the second dose. The median time interval between vaccination and neurological symptoms was 14 days which ranged from 12 h to 63 days. Twelve (22.2 %) patients experienced symptoms of muscle weakness, ten (18.5 %) presented unconsciousness, nine (16.6 %) patients had urinary complaints, nine (16.6 %) had visual impairments, and five (9.2 %) experienced a seizure. After treatments, four (13.8 %) patients died. Forty-six patients had clinical improvement (85.1 %), also improvement in brain MRI was observed among 44 (81.4 %) patients. CONCLUSION: In conclusion, it is not clear that ADEM could be a potential complication of COVID-19 vaccination based on the current evidence and further studies are needed. However, this rare condition should not trigger stopping the mass vaccination programs since the only way to eradicate the current pandemic of COVID-19 is to extend the number of immunized people.


Assuntos
Vacinas contra COVID-19 , COVID-19 , Encefalomielite Aguda Disseminada , Humanos , COVID-19/prevenção & controle , COVID-19/complicações , Vacinas contra COVID-19/efeitos adversos , Encefalomielite Aguda Disseminada/etiologia , Encefalomielite Aguda Disseminada/diagnóstico , Estudos Observacionais como Assunto , SARS-CoV-2 , Vacinação/efeitos adversos
13.
Arch. Soc. Esp. Oftalmol ; 98(2): 116-120, feb. 2023. ilus
Artigo em Espanhol | IBECS | ID: ibc-215180

RESUMO

La encefalomielitis diseminada aguda es una enfermedad inflamatoria-desmielinizante inmunomediada que suele manifestarse tras una infección o vacunación en niños en edad escolar. Típicamente presenta una fase prodrómica con un cuadro pseudogripal seguida de una fase con clínica muy variada, pudiendo aparecer alteraciones neurooftalmológicas como oftalmoplejía o neuritis óptica.La etiología es variada, incluyendo enfermedades tumorales, vasculares, infecciosas, inflamatorias y desmielinizantes. El diagnóstico se basa en la historia clínica y en las características de la resonancia magnética cerebral, prueba de imagen de elección. El estudio del líquido cefalorraquídeo puede servir de ayuda en la orientación del cuadro clínico.El pronóstico es favorable, con excelente respuesta a los corticoides e inmunoglobulinas y con mínimas secuelas a largo plazo en la mayoría de los casos.Presentamos el caso de un varón de 8años con enfermedad desmielinizante aguda por adenovirus cuya manifestación fue un síndrome del ocho y medio. (AU)


Acute disseminated encephalomyelitis is an immune mediated inflammatory-demyelinizing disease that usually manifests after infection or vaccination in school-age children. It typically presents a prodromal phase with flu-like symptoms, followed by a phase with varied clinical symptoms, neuro-ophthalmological alterations such as ophthalmoplegia or optic neuritis may occur.The differential diagnosis includes tumor, vascular, infectious, inflammatory and demyelinating diseases. Diagnosis is based on the clinical history and the characteristics of brain magnetic resonance imaging, the gold standard test. The study of the cerebrospinal fluid can help to guide the clinical picture.The prognosis is favorable, with an excellent response to corticosteroids and immunoglobulins, with minimal long-term sequelae in most cases.We report the case of an 8-year-old male with acute demyelinating disease due to adenovirus whose manifestation was an eight-and-a-half syndrome. (AU)


Assuntos
Humanos , Masculino , Criança , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/virologia , Oftalmoplegia/virologia , Infecções por Adenoviridae/complicações , Tomografia Computadorizada por Raios X , Imageamento por Ressonância Magnética , Síndrome
14.
Emerg Radiol ; 30(1): 127-131, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36480080

RESUMO

Monkeypox is a zoonosis caused by a double-stranded DNA virus of the Poxviridae family. It currently represents a global epidemic given its contagion reported in more than 31 previously non-endemic countries. We present the case of a 30-year-old male patient from Peru with a diagnosis of monkeypox by PCR test, who manifested an initial clinical picture of asthenia, adynamia, and odynophagia, with the appearance of pustular lesions on the lower lip and in the genital region associated with motor and sensory deficit of the lower limbs with altered state of consciousness, with subsequent findings of acute disseminated encephalitis by brain magnetic resonance imaging. This entity corresponds to an infrequent complication with only one case reported in the literature. The importance lies in knowing the possible imaging findings to suspect the diagnosis and expand the knowledge about this current disease.


Assuntos
Encefalomielite Aguda Disseminada , Masculino , Humanos , Adulto , Encefalomielite Aguda Disseminada/diagnóstico , Encefalomielite Aguda Disseminada/etiologia , Encefalomielite Aguda Disseminada/patologia , /patologia , Encéfalo/patologia , Doença Aguda , Imageamento por Ressonância Magnética
16.
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
17.
Zh Nevrol Psikhiatr Im S S Korsakova ; 122(7. Vyp. 2): 60-67, 2022.
Artigo em Russo | MEDLINE | ID: mdl-35912558

RESUMO

At the onset, multiple sclerosis (MS) in children is characterized by a phase of active inflammation with extensive demyelination of the white matter of the CNS, which often mimics this nosology with a whole spectrum of inflammatory demyelinating diseases such as ADEM, anti-MOG encephalitis, ONMSD. In order to formalize the diagnostic process, the International Pediatric Multiple Sclerosis Study Group (IPMSSG) proposed diagnostic criteria for the main immune-mediated demyelinating diseases of the CNS in children, which, in theory, should simplify the diagnostic search. However, in practice, the range of nosologies in the course of differential diagnosis is much wider and often it is not possible to establish an unambiguous diagnosis in the early stages. The authors analyze their own clinical observations based on a sample of 100 pediatric patients with a referral diagnosis of «MS?¼ and describe a clinical case of the onset of highly active MS with an assessment of the dynamics of the clinical and paraclinical course of the disease.


Assuntos
Encefalomielite Aguda Disseminada , Esclerose Múltipla , Criança , Diagnóstico Diferencial , Encefalomielite Aguda Disseminada/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Esclerose Múltipla/diagnóstico
18.
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
19.
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
20.
Acta Biomed ; 93(S1): e20222140, 2022 06 29.
Artigo em Inglês | MEDLINE | ID: mdl-35765956

RESUMO

Different neurological complications associated with the severe acute respiratory syndrome coronavirus (SARS-CoV-2) infection have been widely documented. Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated demyelinating disorder, described within the spectrum of neurological manifestations of COVID-19. Herein, we describe a case of adult-ADEM presenting with diplopia and slowly progressive ataxia developed one month after SARS-CoV-2 infection. Brain magnetic resonance imaging (MRI) revealed acute multifocal demyelinating lesions throughout the brain. Other possible etiologies have been ruled out. After treatment with high-dose steroids, we observed a progressive clinical and radiological improvement. A 4-months follow-up showed complete clinical recovery. Although extremely rare, ADEM could be associated to SARS-CoV-2 infection and should be considered in the differential diagnosis. Early recognition of this COVID-19 neurological complication, even in the absence of pulmonary involvement, is important to start a prompt immune-modulatory treatment and, consequently, ensure a good outcome.


Assuntos
COVID-19 , Encefalomielite Aguda Disseminada , Doenças do Sistema Nervoso , Adulto , Encéfalo/patologia , COVID-19/complicações , Encefalomielite Aguda Disseminada/complicações , Encefalomielite Aguda Disseminada/diagnóstico , Humanos , Doenças do Sistema Nervoso/complicações , SARS-CoV-2
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