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1.
Neurology ; 102(7): e209199, 2024 Apr 09.
Artigo em Inglês | MEDLINE | ID: mdl-38447115

RESUMO

OBJECTIVES: To assess the clinical significance of myelin oligodendrocyte glycoprotein antibodies (MOG-abs) restricted to CSF in children with inflammatory CNS disorders. METHODS: Patients included 760 children (younger than 18 years) from 3 multicenter prospective cohort studies: (A) acquired demyelinating syndromes, including acute disseminated encephalomyelitis (ADEM); (B) non-ADEM encephalitis; and (C) noninflammatory neurologic disorders. For all cases, paired serum/CSF samples were systematically examined using brain immunohistochemistry and live cell-based assays. RESULTS: A total of 109 patients (14%) had MOG-abs in serum or CSF: 79 from cohort A, 30 from B, and none from C. Of these, 63 (58%) had antibodies in both samples, 37 (34%) only in serum, and 9 (8%) only in CSF. Children with MOG-abs only in CSF were older than those with MOG-abs only in serum or in both samples (median 12 vs 6 vs 5 years, p = 0.0002) and were more likely to have CSF oligoclonal bands (86% vs 12% vs 7%, p = 0.0001) and be diagnosed with multiple sclerosis (6/9 [67%] vs 0/37 [0%] vs 1/63 [2%], p < 0.0001). DISCUSSION: Detection of MOG-abs in serum or CSF is associated with CNS inflammatory disorders. Children with MOG-abs restricted to CSF are more likely to have CSF oligoclonal bands and multiple sclerosis than those with MOG-abs detectable in serum.


Assuntos
Doenças do Sistema Nervoso Central , Encefalomielite Aguda Disseminada , Esclerose Múltipla , Criança , Humanos , Bandas Oligoclonais , Estudos Prospectivos , Anticorpos
2.
Hum Vaccin Immunother ; 20(1): 2311969, 2024 Dec 31.
Artigo em Inglês | MEDLINE | ID: mdl-38299507

RESUMO

Acute disseminated encephalomyelitis (ADEM) has been identified as an Adverse Event of Special Interest in the COVID-19 vaccine programme due to its long-standing temporal association with a wide range of other vaccines. Case reports of ADEM shortly following COVID-19 vaccination have now been documented. There were 217 ADEM admissions in 215 individuals in the period 8th December 2020 to 31st March 2023. An increased risk of ADEM following the first dose of ChAdOx1 vaccine was observed (relative incidence (RI) = 3.13, 95% Confidence Interval (CI) [1.56-6.25]) with a vaccine attributable risk of 0.39 per million doses. When doses 1 and 2 were combined this increased risk remained just significant (1.96 [95%CI 1.01-3.82]). No significant increased risk was observed with any other vaccine or dose. This small, elevated risk after the first dose of ChAdOx1-S vaccine demonstrates how large national electronic datasets can be used to identify very rare risks and provides reassurance that any risk of ADEM following the ChAdOx1-S COVID-19 vaccination is extremely small. Given the rarity of this risk, further studies in settings with access to data on large populations should be carried out to verify these findings.


Assuntos
COVID-19 , Encefalomielite Aguda Disseminada , Vacinas , Humanos , Encefalomielite Aguda Disseminada/induzido quimicamente , Encefalomielite Aguda Disseminada/epidemiologia , Vacinas contra COVID-19/efeitos adversos , COVID-19/epidemiologia , COVID-19/prevenção & controle , COVID-19/complicações , Vacinas/efeitos adversos , Vacinação/efeitos adversos , ChAdOx1 nCoV-19 , Inglaterra/epidemiologia
3.
Front Immunol ; 15: 1344184, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38375477

RESUMO

Background: COVID-19 vaccines have been approved due to their excellent safety and efficacy data and their use has also permitted to reduce neurological complications of SARS-CoV-2. However, clinical trials were underpowered to detect rare adverse events. Herein, the aim was to characterize the clinical spectrum and immunological features of central nervous system (CNS) immune-related events following SARS-CoV-2 vaccination. Methods: Multicenter, retrospective, cohort study (December 1, 2020-April 30, 2022). Inclusion criteria were (1) de novo CNS disorders developing after SARS-CoV-2 vaccination (probable causal relationship as per 2021 Butler criteria) (2); evidence for an immune-mediated etiology, as per (i) 2016 Graus criteria for autoimmune encephalitis (AE); (ii) 2015 Wingerchuk criteria for neuromyelitis optica spectrum disorders; (iii) criteria for myelitis. Results: Nineteen patients were included from 7 tertiary referral hospitals across Italy and France (one of them being a national referral center for AE), over almost 1 year and half of vaccination campaign. Vaccines administered were mRNA-based (63%) and adenovirus-vectored (37%). The median time between vaccination and symptoms onset was 14 days (range: 2-41 days). CSF was inflammatory in 74%; autoantibodies were detected in 5%. CSF cytokine analysis (n=3) revealed increased CXCL-10 (IP-10), suggesting robust T-cell activation. The patients had AE (58%), myelitis (21%), acute disseminated encephalomyelitis (ADEM) (16%), and brainstem encephalitis (5%). All patients but 2 received immunomodulatory treatment. At last follow-up (median 130 days; range: 32-540), only one patient (5%) had a mRS>2. Conclusion: CNS adverse events of COVID-19 vaccination appear to be very rare even at reference centers and consist mostly of antibody-negative AE, myelitis, and ADEM developing approximately 2 weeks after vaccination. Most patients improve following immunomodulatory treatment.


Assuntos
COVID-19 , Encefalomielite Aguda Disseminada , Mielite , Neuromielite Óptica , Humanos , SARS-CoV-2 , Vacinas contra COVID-19/efeitos adversos , COVID-19/prevenção & controle , Estudos Retrospectivos , Estudos de Coortes , Vacinação/efeitos adversos , Neuromielite Óptica/terapia , Encefalomielite Aguda Disseminada/etiologia , Sistema Nervoso Central
4.
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
5.
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
6.
Neurol Sci ; 45(3): 1263-1266, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38177968

RESUMO

Saint Louis encephalitis virus (SLEV) infection is an arbovirosis associated with a broad spectrum of neurological complications. We present a case of a 55-year-old man hailing from Manaus, a city situated in the heart of the Amazon Rainforest, who exhibited symptoms of vertigo, tremors, urinary and fecal retention, compromised gait, and encephalopathy 3 weeks following SLEV infection. Neuroaxis MRI revealed diffuse, asymmetric, and poorly defined margins hyperintense lesions with peripheral and ring enhancement in subcortical white matter, as well as severe spinal cord involvement. Serology for SLEV was positive both on serum and cerebrospinal fluid. To the best of our knowledge, the present report is the first to show brain lesions along with myelitis as a post-infectious complication of SLEV infection.


Assuntos
Encefalite de St. Louis , Encefalomielite Aguda Disseminada , Masculino , Humanos , Pessoa de Meia-Idade , Vírus da Encefalite de St. Louis/fisiologia , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/etiologia , Encefalite de St. Louis/complicações , Encefalite de St. Louis/diagnóstico
7.
Semin Pediatr Neurol ; 47: 101078, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37919033

RESUMO

MEASLES VIRUS AND ASSOCIATED CENTRAL NERVOUS SYSTEM: Sequelae Renee Buchanan, Daniel J. Bonthius Seminars in Pediatric Neurology Volume 19, Issue 3, September 2012, Pages 107-114 Worldwide, measles remains one of the most deadly vaccine-preventable diseases. In the United States, enrollment in the public schools requires that each child receives 2 doses of measles-containing vaccine before entry, essentially eliminating this once endemic disease. Recent outbreaks of measles in the United States have been associated with importation of measles virus from other countries and subsequent transmission to intentionally undervaccinated children. The central nervous system complications of measles can occur within days or years of acute infection and are often severe. These include primary measles encephalitis, acute postinfectious measles encephalomyelitis, measles inclusion body encephalitis, and subacute sclerosing panencephalitis. These measles associated central nervous system diseases differ in their pathogenesis and pathologic effects. However, all involve complex brain-virus-immune system interactions, and all can lead to severe and permanent brain injury. Despite better understanding of the clinical presentations and pathogenesis of these illnesses, effective treatments remain elusive.


Assuntos
Encefalomielite Aguda Disseminada , Sarampo , Panencefalite Esclerosante Subaguda , Criança , Humanos , Vírus do Sarampo/fisiologia , Sistema Nervoso Central , Sarampo/complicações , Sarampo/epidemiologia , Sarampo/prevenção & controle , Panencefalite Esclerosante Subaguda/epidemiologia , Panencefalite Esclerosante Subaguda/terapia , Panencefalite Esclerosante Subaguda/complicações , Encéfalo , Encefalomielite Aguda Disseminada/complicações
8.
J Med Case Rep ; 17(1): 457, 2023 Oct 17.
Artigo em Inglês | MEDLINE | ID: mdl-37845760

RESUMO

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently described autoimmune inflammatory disorder of the central nervous system (CNS). There is limited data on the association between Human Immunodeficiency virus (HIV) infection and MOGAD. We report three patients with HIV infection and myelin oligodendrocyte glycoprotein (MOG) antibodies in the setting of other central nervous system infections. CASE DESCRIPTIONS: The first patient, a 44-year-old black African man, presented with acute disseminated encephalomyelitis (ADEM) with positive serum MOG antibodies. He made a significant recovery with corticosteroids but had a quick relapse and died from sepsis. The second patient, an 18-year-old black woman, presented with paraplegia and imaging revealed a longitudinally extensive transverse myelitis and had positive serum MOG antibodies. She remained paraplegic after methylprednisone and plasmapheresis treatments. Her rehabilitation was complicated by development of pulmonary embolism and tuberculosis. The third patient, a 43-year-old mixed-race woman, presented with bilateral painless visual loss. Her investigations were notable for positive MOG antibodies, positive Varicella Zoster Virus on cerebral spinal fluid (CSF) and hyperintense optic nerves on magnetic resonance imaging (MRI). Her vision did not improve with immunosuppression and eventually died from sepsis. CONCLUSION: Our cases illustrate the diagnostic and management challenges of MOGAD in the setting of advanced HIV infection, where the risk of CNS opportunistic infections is high even without the use of immunosuppression. The atypical clinical progression and the dilemmas in the diagnosis and treatment of these cases highlight gaps in the current knowledge of MOGAD among people with HIV that need further exploration.


Assuntos
Encefalomielite Aguda Disseminada , Infecções por HIV , Sepse , Masculino , Feminino , Humanos , Adulto , Adolescente , Glicoproteína Mielina-Oligodendrócito , Infecções por HIV/complicações , Infecções por HIV/tratamento farmacológico , Autoanticorpos
9.
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
10.
Zh Nevrol Psikhiatr Im S S Korsakova ; 123(7. Vyp. 2): 116-121, 2023.
Artigo em Russo | MEDLINE | ID: mdl-37560843

RESUMO

Acute hemorrhagic leukoencephalitis (AHLE), also called Hurst's encephalitis, is a rare demyelinating disease of the central nervous system characterized by rapid progression and acute inflammation of the white matter of the brain and spinal cord. AHLE is currently considered as a rare, most severe variant of acute disseminated encephalomyelitis. Clinically AHLE is characterized by a fulminant course with a rapid development of encephalopathy and multifocal neurological symptoms. AHLE is associated with high mortality rate that requires immediate and aggressive treatment initiation. This article describes a case of AHLE with an atypical course, a subacute form, which is extremely rarely described in the literature, with the progressive symptoms' development over several months. Due to delayed treatment initiation, unfortunately, a fatal outcome has been observed. Subsequent histological examination of the autopsy material confirmed the presence of a subacute form of AHLE in the patient.


Assuntos
Encefalite , Encefalomielite Aguda Disseminada , Leucoencefalite Hemorrágica Aguda , Humanos , Leucoencefalite Hemorrágica Aguda/diagnóstico , Leucoencefalite Hemorrágica Aguda/patologia , Encéfalo/patologia , Medula Espinal , Encefalite/patologia , Imageamento por Ressonância Magnética
11.
Am J Med Genet A ; 191(12): 2868-2872, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37589194

RESUMO

Thiamine pyrophosphate (TPP), the substrate of Thiamine pyrophosphate kinase (TPK), is an important cofactor in carbohydrate metabolism, specifically as a cofactor of the Pyruvate dehydrogenase complex (PDH) complex. The nervous system is particularly dependent on TPP due to its reliance on glucose metabolism. In this case, a four-year-old girl had a previously unreported pathogenic variant of the gene encoding TPK (TPK1) which presented as Thiamine metabolism dysfunction syndrome 5 (THMD5; OMIM 614458). She had been diagnosed with acute disseminated encephalomyelitis and autism spectrum disorder (ASD), and initially presented with fever and agitation following vaccinations. After follow-up with genetic testing, our patient was found to have compound heterozygous pathogenic variants of TPK1. After treatment with biotin and thiamine her clinical status improved, and her ASD features resolved. The presentation of our patient was consistent with previous reports and adds to the evidence that thiamine and biotin are effective treatments of TPK1 related metabolic deficiencies. The improvement of neurobehavioral symptoms in this case was marked, highlighting the importance of early identification and therapeutic intervention in this condition.


Assuntos
Transtorno do Espectro Autista , Encefalomielite Aguda Disseminada , Humanos , Feminino , Pré-Escolar , Encefalomielite Aguda Disseminada/tratamento farmacológico , Biotina/uso terapêutico , Tiamina/uso terapêutico , Tiamina/genética , Tiamina/metabolismo , Tiamina Pirofosfato/metabolismo
12.
J Child Neurol ; 38(8-9): 537-549, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37574786

RESUMO

AIM: We reviewed the clinical features of a sample of pediatric acquired demyelinating syndromes with the purpose of determining the appropriate protocol for follow-up after the first episode. METHODS: A multicenter retrospective observational study was conducted on a cohort of 40 children diagnosed with a first episode of acquired demyelinating syndrome over the period 2012-2021. Patients were evaluated with clinical and neuroradiologic assessment after 3, 6, and 12 months, with a median follow-up of 4.0 years. RESULTS: At the first acquired demyelinating syndrome episode, 18 patients (45%) were diagnosed with acute disseminated encephalomyelitis, 18 (45%) with clinical isolated syndrome, and 4 (10%) with multiple sclerosis. By month 12, 12 patients (30%) had progressed from an initial diagnosis of acute disseminated encephalomyelitis (2) or clinical isolated syndrome (10) to multiple sclerosis. Of these, 6 had clinical relapse and 6 radiologic relapse only. The first relapse occurred after a median of 3 months. Among the patients who had evolved toward multiple sclerosis, there was a prevalence of females (P = .014), higher oligoclonal bands positivity (P = .009), and older median age (P < .001) as compared with those who had remained stable. INTERPRETATION: Both clinical and radiologic follow-up of children with acquired demyelinating syndromes is crucial, especially during the first year after acute onset, for early identification of multiple sclerosis and prompt initiation of disease-modifying treatment to delay axonal damage and to limit disability.


Assuntos
Encefalomielite Aguda Disseminada , Esclerose Múltipla , Feminino , Criança , Humanos , Masculino , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Encefalomielite Aguda Disseminada/terapia , Seguimentos , Síndrome , Sistema Nervoso Central , Esclerose Múltipla/diagnóstico por imagem , Esclerose Múltipla/epidemiologia , Recidiva , Estudos Observacionais como Assunto , Estudos Multicêntricos como Assunto
13.
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
14.
Mult Scler ; 29(9): 1080-1089, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37431144

RESUMO

BACKGROUND: The potential therapeutic benefit of intravenous immunoglobulins (IVIGs) for acute attacks of myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is unknown. OBJECTIVE: The objective was to describe the outcomes of IVIG treatment for acute MOGAD attacks. METHODS: A retrospective observational study involving seven tertiary neuroimmunology centers. Data collection included patients' demographics, Expanded Disability Status Scale (EDSS), and visual acuity (VA) before the attack, at the nadir of the attack before IVIG treatment, and at follow-up visits ⩾3 months after treatment. RESULTS: Thirty-nine patients were included, of which 21 (53.8%) were female. The median age was 23 years (range 5-74 years), and the median disease duration was 4 months (range 0-93 months). The most common type of attack treated with IVIG was isolated optic neuritis (ON) (unilateral n = 14, bilateral n = 5, associated with transverse myelitis (TM), n = 1), followed by acute disseminated encephalomyelitis (ADEM) (n = 8), multifocal (n = 7), TM (n = 3), brainstem (n = 1), and other encephalitis (n = 1). A significant improvement in both the EDSS and VA measures was observed at follow-up compared to the time of IVIG treatment initiation (p < 0.0001 for both outcome measures). CONCLUSION: IVIG may be an effective treatment option for acute MOGAD attacks. Further prospective studies are warranted to validate our results.


Assuntos
Encefalomielite Aguda Disseminada , Mielite Transversa , Neuromielite Óptica , Feminino , Masculino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Glicoproteína Mielina-Oligodendrócito , Autoanticorpos , Encefalomielite Aguda Disseminada/tratamento farmacológico , Estudos Retrospectivos
15.
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
17.
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
18.
J Neuroimaging ; 33(5): 752-763, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37381160

RESUMO

BACKGROUND AND PURPOSE: To determine the incidence of acute neuroimaging (NI) findings and comorbidities in the coronavirus disease of 2019 (COVID-19)-infected subjects in seven U.S. and four European hospitals. METHODS: This is a retrospective study of COVID-19-positive subjects with the following inclusion criteria: age >18, lab-confirmed COVID-19 infection, and acute NI findings (NI+) attributed to COVID-19 on CT or MRI brain. NI+ and comorbidities in total hospitalized COVID-19-positive (TN) subjects were assessed. RESULTS: A total of 37,950 COVID-19-positive subjects were reviewed and 4342 underwent NI. NI+ incidence in subjects with NI was 10.1% (442/4342) including 7.9% (294/3701) in the United States and 22.8% (148/647) in Europe. NI+ incidence in TN was 1.16% (442/37,950). In NI (4342), incidence of ischemic stroke was 6.4% followed by intracranial hemorrhage (ICH) (3.8%), encephalitis (0.5%), sinus venous thrombosis (0.2%), and acute disseminated encephalomyelitis (ADEM) (0.2%). White matter involvement was seen in 57% of NI+. Hypertension was the most common comorbidity (54%) before cardiac disease (28.8%) and diabetes mellitus (27.7%). Cardiac disease (p < .025), diabetes (p < .014), and chronic kidney disease (p < .012) were more common in the United States. CONCLUSION: This multicenter, multinational study investigated the incidence and spectrum of NI+ in 37,950 hospitalized adult COVID-19 subjects including regional differences in incidences of NI+, associated comorbidities, and other demographics. NI+ incidence in TN was 1.16% including 0.95% in the United States and 2.09% in Europe. ICH, encephalitis, and ADEM were common in Europe, while ischemic strokes were more common in the United States. In this cohort, incidence and distribution of NI+ helped characterize the neurological complications of COVID-19.


Assuntos
COVID-19 , Encefalite , Encefalomielite Aguda Disseminada , Cardiopatias , AVC Isquêmico , Adulto , Humanos , Estados Unidos/epidemiologia , COVID-19/diagnóstico por imagem , COVID-19/epidemiologia , Estudos Retrospectivos , Neuroimagem/métodos , Hemorragias Intracranianas , Europa (Continente)/epidemiologia
19.
Mult Scler Relat Disord ; 76: 104787, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37320939

RESUMO

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently described neuroinflammatory demyelinating disease. OBJECTIVE: To better understand the clinical spectrum, risk factors and outcomes in MOGAD. METHODS: Retrospective cohort study including all subjects harboring anti-MOG antibodies identified in major academic hospitals across the province of Quebec. RESULTS: We identified 45 MOGAD cases. The minimal estimated point-prevalence was 0.52/100 000 in Quebec. Median age at presentation was 32 years (range 1-71) with equal sex ratio. Most frequent ethnic groups were Caucasians and Asians. The most frequent clinical manifestations at onset were optic neuritis (ON), affecting 56% of adults, and acute disseminated encephalomyelitis (ADEM), affecting 33% of children. First MRI was abnormal in 84% of cases. Most CSF samples showed pleocytosis without oligoclonal bands. Two brain biopsies revealed lipid-laden macrophages and reactive astrocytes. Despite steroids, only 38% had fully recovered at 4 weeks after onset. Half of pediatric and two thirds of adult-onset MOGAD subjects experienced relapses. At last follow-up, 69% showed residual deficits, which were moderate to severe in 17% of adults. CONCLUSION: MOGAD has heterogeneous disease course, and it is not a benign disease for a substantial proportion of adults. Best disease-modifying therapies remain to be determined.


Assuntos
Encefalomielite Aguda Disseminada , Neurite Óptica , Humanos , Glicoproteína Mielina-Oligodendrócito , Estudos Retrospectivos , Encefalomielite Aguda Disseminada/diagnóstico por imagem , Progressão da Doença , Autoanticorpos
20.
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
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