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1.
Pediatr Neurol ; 155: 126-132, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38636169

ABSTRACT

BACKGROUND: Various factors contribute to the development of infection-related acute encephalopathy (AE) in children, such as infectious agents and chronic underlying disorders. We studied underlying disorders in children with AE to identify predisposing factors of AE. METHODS: We investigated underlying disorders or past histories in patients with two types of AE from the database in the Tokai area of Japan between 2009 and 2022: 204 patients with AE with reduced subcortical diffusion (AED) and 137 with clinically mild encephalopathy with a reversible splenial lesion (MERS). We compared them with 89 patients with acute disseminated encephalomyelitis (ADEM) to clarify the specific disorders in the two AE types. RESULTS: The prevalence of underlying disorders in AED (34%, 70 patients) was significantly higher than that in ADEM (12%, 11 patients) (P < 0.01). The prevalence of underlying disorders in MERS was 23% (32 patients). The underlying disorders included seizure disorders, premature birth, genetic/congenital disorders, and endocrine/renal diseases. In patients with seizure disorders in AED, five patients (18%) had Dravet syndrome and four (15%) had West syndrome, whereas none with MERS had these syndromes. Twenty-five (12%) of 204 patients with AED, three (2%) with MERS, and one (1%) with ADEM were preterm or low birth weight. CONCLUSIONS: The high prevalence of seizure disorders suggests that seizure susceptibility is an important predisposing factor in AED. Premature birth also has an impact on the development of AED. Caution is required regarding the development of AE in patients with chronic seizure disorders or premature birth.


Subject(s)
Brain Diseases , Humans , Male , Female , Child, Preschool , Infant , Child , Brain Diseases/epidemiology , Brain Diseases/etiology , Brain Diseases/complications , Adolescent , Japan/epidemiology , Prevalence , Infant, Newborn , Encephalomyelitis, Acute Disseminated/epidemiology , Encephalomyelitis, Acute Disseminated/etiology , Encephalomyelitis, Acute Disseminated/complications
2.
Hum Vaccin Immunother ; 20(1): 2311969, 2024 Dec 31.
Article in English | MEDLINE | ID: mdl-38299507

ABSTRACT

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.


Subject(s)
COVID-19 , Encephalomyelitis, Acute Disseminated , Vaccines , Humans , Encephalomyelitis, Acute Disseminated/chemically induced , Encephalomyelitis, Acute Disseminated/epidemiology , COVID-19 Vaccines/adverse effects , COVID-19/epidemiology , COVID-19/prevention & control , COVID-19/complications , Vaccines/adverse effects , Vaccination/adverse effects , ChAdOx1 nCoV-19 , England/epidemiology
3.
Vaccine ; 42(9): 2212-2219, 2024 Apr 02.
Article in English | MEDLINE | ID: mdl-38350771

ABSTRACT

Acute Disseminated Encephalomyelitis (ADEM) and Transverse Myelitis (TM) are within the group of immune mediated disorders of acquired demyelinating syndromes. Both have been described in temporal association following various vaccinations in case reports and case series and have been evaluated in observational studies. A recent analysis conducted by The Global Vaccine Data Network (GVDN) observed an excess of ADEM and TM cases following the adenoviral vectored ChAdOx1 nCoV-19 (AZD1222) and mRNA-1273 vaccines, compared with historically expected background rates from prior to the pandemic. Further epidemiologic studies were recommended to explore these potential associations. We utilized an Australian vaccine datalink, Vaccine Safety Health-Link (VSHL), to perform a self-controlled case series analysis for this purpose. VSHL was selected for this analysis as while VSHL data are utilised for GVDN association studies, they were not included in the GVDN observed expected analyses. The VSHL dataset contains vaccination records sourced from the Australian Immunisation Register, and hospital admission records from the Victorian Admitted Episodes Dataset for 6.7 million people. These datasets were used to determine the relative incidence (RI) of G040 (ADEM) and G373 (TM) ICD-10-AM coded admissions in the 42-day risk window following COVID-19 vaccinations as compared to control periods either side of the risk window. We observed associations between ChAdOx1 adenovirus vector COVID-19 vaccination and ADEM (all dose RI: 3.74 [95 %CI 1.02,13.70]) and TM (dose 1 RI: 2.49 [95 %CI: 1.07,5.79]) incident admissions. No associations were observed between mRNA COVID-19 vaccines and ADEM or TM. These findings translate to an extremely small absolute risk of ADEM (0.78 per million doses) and TM (1.82 per million doses) following vaccination; any potential risk of ADEM or TM should be weighed against the well-established protective benefits of vaccination against COVID-19 disease and its complications. This study demonstrates the value of the GVDN collaboration leveraging large population sizes to examine important vaccine safety questions regarding rare outcomes, as well as the value of linked population level datasets, such as VSHL, to rapidly explore associations that are identified.


Subject(s)
COVID-19 , Encephalomyelitis, Acute Disseminated , Myelitis, Transverse , Vaccines , Humans , Australia/epidemiology , ChAdOx1 nCoV-19 , COVID-19/epidemiology , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Encephalomyelitis, Acute Disseminated/chemically induced , Encephalomyelitis, Acute Disseminated/epidemiology , Myelitis, Transverse/etiology , Myelitis, Transverse/complications , Vaccination/adverse effects
4.
Pharmazie ; 77(7): 262-269, 2022 09 01.
Article in English | MEDLINE | ID: mdl-36199182

ABSTRACT

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


Subject(s)
Encephalomyelitis, Acute Disseminated , Influenza Vaccines , Influenza, Human , Adverse Drug Reaction Reporting Systems , Encephalomyelitis, Acute Disseminated/chemically induced , Encephalomyelitis, Acute Disseminated/epidemiology , Humans , Influenza Vaccines/adverse effects , Influenza, Human/epidemiology , Influenza, Human/prevention & control , Seasons , United States/epidemiology , Vaccination/adverse effects
5.
Vaccine ; 40(24): 3305-3312, 2022 05 26.
Article in English | MEDLINE | ID: mdl-35527057

ABSTRACT

BACKGROUND: Background incidence rates are critical in pharmacovigilance to facilitate identification of vaccine safety signals. We estimated background incidence rates of 11 adverse events of special interest related to COVID-19 vaccines in Ontario, Canada. METHODS: We conducted a population-based retrospective observational study using linked health administrative databases for hospitalizations and emergency department visits among Ontario residents. We estimated incidence rates of Bell's palsy, idiopathic thrombocytopenia, febrile convulsions, acute disseminated encephalomyelitis, myocarditis, pericarditis, Kawasaki disease, Guillain-Barré syndrome, transverse myelitis, acute myocardial infarction, and anaphylaxis during five pre-pandemic years (2015-2019) and 2020. RESULTS: The average annual population was 14 million across all age groups with 51% female. The pre-pandemic mean annual rates per 100,000 population during 2015-2019 were 191 for acute myocardial infarction, 43.9 for idiopathic thrombocytopenia, 28.8 for anaphylaxis, 27.8 for Bell's palsy, 25.0 for febrile convulsions, 22.8 for acute disseminated encephalomyelitis, 11.3 for myocarditis/pericarditis, 8.7 for pericarditis, 2.9 for myocarditis, 2.0 for Kawasaki disease, 1.9 for Guillain-Barré syndrome, and 1.7 for transverse myelitis. Females had higher rates of acute disseminated encephalomyelitis, transverse myelitis and anaphylaxis while males had higher rates of myocarditis, pericarditis, and Guillain-Barré syndrome. Bell's palsy, acute disseminated encephalomyelitis, and Guillain-Barré syndrome increased with age. The mean rates of myocarditis and/or pericarditis increased with age up to 79 years; males had higher rates than females: from 12 to 59 years for myocarditis and ≥12 years for pericarditis. Febrile convulsions and Kawasaki disease were predominantly childhood diseases and generally decreased with age. CONCLUSIONS: Our estimated background rates will permit estimating numbers of expected events for these conditions and facilitate detection of potential safety signals following COVID-19 vaccination.


Subject(s)
COVID-19 Vaccines , COVID-19 , Anaphylaxis/chemically induced , Anaphylaxis/epidemiology , Bell Palsy/chemically induced , Bell Palsy/epidemiology , COVID-19/epidemiology , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Encephalomyelitis, Acute Disseminated/chemically induced , Encephalomyelitis, Acute Disseminated/epidemiology , Female , Guillain-Barre Syndrome/chemically induced , Guillain-Barre Syndrome/epidemiology , Humans , Incidence , Male , Mucocutaneous Lymph Node Syndrome/chemically induced , Mucocutaneous Lymph Node Syndrome/epidemiology , Myelitis, Transverse/chemically induced , Myelitis, Transverse/epidemiology , Myocardial Infarction/chemically induced , Myocardial Infarction/epidemiology , Myocarditis/chemically induced , Myocarditis/epidemiology , Ontario/epidemiology , Pericarditis/chemically induced , Pericarditis/epidemiology , Purpura, Thrombocytopenic, Idiopathic/chemically induced , Retrospective Studies , Seizures, Febrile/chemically induced , Seizures, Febrile/epidemiology
6.
Zh Nevrol Psikhiatr Im S S Korsakova ; 121(11): 119-128, 2021.
Article in Russian | MEDLINE | ID: mdl-34932297

ABSTRACT

Acute disseminated encephalomyelitis (AEM) is an immune-mediated inflammatory demyelinating disease of the central nervous system (CNS), usually single-phase. In WREM, multiple lesions of the central nervous system of an inflammatory-demyelinating nature accompanied by extremely polymorphic neurological disorders develop acutely or subacutely. The review considers the issues of epidemiology, trigger factors of the inflammatory process, clinical manifestations, differential and molecular diagnostics of WECM, and outlines the ways for further study of this pathology.


Subject(s)
Encephalomyelitis, Acute Disseminated , Encephalomyelitis , Multiple Sclerosis , Brain , Central Nervous System , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/epidemiology , Humans
7.
Arch Pediatr ; 28(8): 638-646, 2021 Nov.
Article in English | MEDLINE | ID: mdl-34690025

ABSTRACT

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder of the central nervous system. Little information is available about the clinical and neuroradiological profile or the follow-up of this disease in Tunisian children. AIM: To determine the clinical, laboratory, and radiological features and the outcome of ADEM in children admitted to the pediatrics department of a university hospital in Tunisia. METHODS: All children ≤ 18 years old presenting with ADEM and admitted to the tertiary referral center for pediatrics at Sahloul University Hospital from January 2000 to December 2020 were included in the study. The diagnosis of ADEM was confirmed according to the international pediatric multiple sclerosis study group criteria. RESULTS: A total of 20 patients (13 girls and 7 boys) fulfilled the diagnostic criteria for ADEM. The mean age at diagnosis was 5.6 years. The clinical presentation included polyfocal neurological signs such as cranial hypertension (45%), seizures (35%), and motor weaknesses (55%). Pyramidal tract signs and cranial nerve palsies were noted in 55% of cases. Brain magnetic resonance imaging showed particular features, namely, a relapsing tumor-like form in one case, and optic neuritis and demyelinating lesions of the white matter in the brain and the spinal cord with gadolinium cerebral ring-like enhancement in another case. The treatment consisted of intravenous immunoglobulin in 16 cases (80%) and corticosteroid in 19 cases (95%). Plasmapheresis was used for one patient. Complete recovery was observed in 12 patients (60%); 19 patients (95%) had a monophasic course of the disease while only one patient developed multiphasic ADEM. CONCLUSIONS: ADEM remains a difficult diagnosis in children. Nevertheless, after prompt diagnosis and adequate treatment, most children with ADEM have a favorable outcome with restitutio ad integrum.


Subject(s)
Encephalomyelitis, Acute Disseminated/diagnosis , Adolescent , Adrenal Cortex Hormones/therapeutic use , Child , Child, Preschool , Encephalomyelitis, Acute Disseminated/classification , Encephalomyelitis, Acute Disseminated/epidemiology , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Infant , Male , Pediatrics/methods , Pediatrics/statistics & numerical data , Retrospective Studies , Tunisia/epidemiology
9.
Hum Vaccin Immunother ; 17(8): 2578-2585, 2021 08 03.
Article in English | MEDLINE | ID: mdl-33835888

ABSTRACT

Acute disseminated encephalomyelitis (ADEM) is an autoimmune, central nervous system demyelinating disorder that follows antecedent immunologic challenges, such as infection or vaccination. This study aimed to investigate the potential association between routine childhood vaccinations and ADEM. Children under 7 years of age admitted to the two tertiary level pediatric hospitals in Victoria, Australia with ADEM from 2000-2015 had their clinical information linked to vaccination records from the Australian Childhood Immunization Register. Chart review was undertaken utilizing the Brighton Collaboration ADEM criteria. The self-controlled case-series (SCCS) methodology was employed to determine the relative incidences of ADEM post-vaccination in two risk intervals: 5-28 days and 2-42 days. Forty-six cases were eligible for SCCS analysis with a median age of 3.2 years. Of the forty-six cases, three were vaccine proximate cases and received vaccinations 23, 25 and 28 days before ADEM onset. Two vaccine proximate cases received their 4-year-old scheduled vaccinations (MMR and DTPa-IPV) and one vaccine proximate case the 1-year old scheduled vaccinations (MMR and Hib-MenC). The relative incidence of ADEM during the narrow and broad risk intervals were 1.041 (95% CI 0.323-3.356, p = 0.946) and 0.585 (95% CI 0.182-1.886, p = 0.370) respectively. Sensitivity analyses did not yield any substantial deviations. These results do not provide evidence of an association between vaccinations routinely provided to children aged under 7 years in Australia and the incidence of ADEM. However, these results should be interpreted with caution as the number of ADEM cases identified was limited and further research is warranted.


Subject(s)
Encephalomyelitis, Acute Disseminated , Vaccines , Child , Child, Preschool , Encephalomyelitis, Acute Disseminated/epidemiology , Humans , Incidence , Infant , Vaccination , Victoria
11.
Acta Neurol Belg ; 121(4): 927-931, 2021 Aug.
Article in English | MEDLINE | ID: mdl-32314270

ABSTRACT

Myelin oligodendrocyte glycoprotein antibody (MOG-Ab) is involved in the pathogenesis of central nervous system (CNS) demyelination disorders. We aimed to explore the spectrum of MOG-Ab-associated diseases in eastern India. A single-center, prospective observational study was done over a period of 2 years in a tertiary care hospital of eastern India. Patients with CNS demyelination disorders who tested positive for MOG-Ab using live cell-based assay were included in the study; while, those with age less than 1 year, documented preexisting CNS structural lesions, developmental delays or diagnosed multiple sclerosis were excluded. Demographic profile, clinical spectrum, disease course, radiological features as well as response to treatment were analyzed among included patients. Twenty MOG-Ab-positive patients were included (M:F 1:1.85). The median age of symptom onset was 10.5 years. The median follow-up of patients was 13 months. Acute disseminated encephalomyelitis (ADEM) was the commonest presentation at first attack (55%), followed by optic neuritis (ON) (45%). Patients with ADEM had a significantly lower age at first attack (p = 0.025). Monophasic and relapsing disease courses were seen in 45% and 55% patients, respectively. While all patients with only ADEM had a monophasic course, 77.8% with ON had a relapsing course. Among patients who presented with isolated transverse myelitis, 75% had a monophasic course and all had disease confined to the spinal cord. Good response to corticosteroids was seen in majority of participants. Second-line drugs were needed in 55% patients, rituximab being the commonest second-line agent used. 35% patients had significant disability (EDSS > 4) at last follow-up. MOG-Ab-associated diseases have diverse clinical phenotypes characterized by age-dependent pattern-specific courses.


Subject(s)
Autoantibodies/blood , Encephalomyelitis, Acute Disseminated/blood , Myelin-Oligodendrocyte Glycoprotein/blood , Myelitis, Transverse/blood , Optic Neuritis/blood , Adolescent , Adult , Child , Encephalomyelitis, Acute Disseminated/diagnostic imaging , Encephalomyelitis, Acute Disseminated/epidemiology , Female , Follow-Up Studies , Humans , India/epidemiology , Male , Myelitis, Transverse/diagnostic imaging , Myelitis, Transverse/epidemiology , Optic Neuritis/diagnostic imaging , Optic Neuritis/epidemiology , Prospective Studies , Young Adult
12.
J Child Neurol ; 36(2): 141-147, 2021 02.
Article in English | MEDLINE | ID: mdl-32988277

ABSTRACT

The epidemiology of demyelinating diseases in the Korean pediatric population has not been reported to date. This study aimed to identify the epidemiology of demyelinating diseases in Korean children by using big data. The subjects were children (0-17 years old) diagnosed with acute-disseminated encephalomyelitis, multiple sclerosis, neuromyelitis optica, and Guillain-Barré syndrome enrolled in the Korean Health Insurance Review and Assessment Service (HIRA) from January 2010 to December 2017.Of 1722 enrolled children, 553 (32.1%) had acute-disseminated encephalomyelitis, 170 (9.9%) had multiple sclerosis, 68 (3.9%) had neuromyelitis optica, and 931 (54.1%) had Guillain-Barré syndrome. The male-female ratios were 1.47:1 in acute-disseminated encephalomyelitis, 1.43:1 in Guillain-Barré syndrome, 1:1.66 in multiple sclerosis, and 1:1.62 in neuromyelitis optica. Demyelinating diseases were most prevalent in summer. The prevalence differed by region, with 545 (31.6%) in Seoul and 298 (17.3%) in Gyeonggi. This study is the first to identify the incidence of demyelinating diseases in South Korea.


Subject(s)
Encephalomyelitis, Acute Disseminated/epidemiology , Guillain-Barre Syndrome/epidemiology , Multiple Sclerosis/epidemiology , Neuromyelitis Optica/epidemiology , Adolescent , Child , Child, Preschool , Encephalomyelitis, Acute Disseminated/physiopathology , Female , Guillain-Barre Syndrome/physiopathology , Humans , Incidence , Infant , Male , Multiple Sclerosis/physiopathology , Neuromyelitis Optica/physiopathology , Republic of Korea/epidemiology
13.
Mult Scler Relat Disord ; 46: 102573, 2020 Nov.
Article in English | MEDLINE | ID: mdl-33059214

ABSTRACT

BACKGROUND: Abnormal thyroid peroxidase antibody (TPOAb) levels are observed in various autoimmune diseases. However, the relationship between TPOAb and pediatric acute disseminated encephalomyelitis (PADEM) remains unclear. This study aimed to investigate the positive rate of TPOAb and thyroid dysfunction in children with acute disseminated encephalomyelitis (ADEM) and assess the relationship between TPOAb and clinical features of PADEM. METHODS: This retrospective single-center case-control study was conducted from April 2017 to April 2019. We enrolled 23 children with ADEM and 23 age- and sex-matched healthy controls. Based on whether they were positive for TPOAb, the children with ADEM were allocated either to the TPOAb+ or TPOAb- group. The median follow-up time was 12 months (6-30 months). Observers were blinded to the patient groupings. We compared the clinical and imaging characteristics of the two groups. RESULTS: Among the 23 patients with PADEM, 47.8% presented with abnormal TPOAb levels, while there were no TPOAb+ cases in the control group. Among the children with ADEM, there were significantly increased TPOAb positive rates and significantly decreased fT3 levels. TPOAb+ and TPOAb- subgroup analysis revealed significant differences in gait, fever, and total IgG. In the TPOAb+ group, there was a significant decrease in TPOAb levels at 2 weeks after ADEM onset. The follow-up of patients who were TPOAb+ at 3 months after onset showed a gradual decrease in their TPOAb levels back to normal. One patient who presented new nervous system symptoms after over 1 month also showed a simultaneous increase in TPOAb levels. There was a significant negative correlation between Glasgow Coma Scale (GCS) scores and TPOAb levels (p = 0.042, r = -0.892). CONCLUSION: There was a negative correlation of TPOAb levels with GCS scores. Therefore, TPOAb levels could be used for the prognosis of patients with PADEM. We recommend determining thyroid function when assessing patients with PADEM during follow-up.


Subject(s)
Encephalomyelitis, Acute Disseminated , Thyroid Gland , Autoantibodies , Autoimmunity , Case-Control Studies , Child , Encephalomyelitis, Acute Disseminated/complications , Encephalomyelitis, Acute Disseminated/diagnostic imaging , Encephalomyelitis, Acute Disseminated/epidemiology , Humans , Retrospective Studies , Thyroid Gland/diagnostic imaging
14.
Mult Scler Relat Disord ; 46: 102424, 2020 Nov.
Article in English | MEDLINE | ID: mdl-32829177

ABSTRACT

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disorder. Most studies involve white children in developed countries in the northern hemisphere. The authors aimed to describe the clinical course and prognostic of a cohort of adult patients with ADEM from Rio de Janeiro city, where most of the population is Afro-descendant. METHODS: We performed a longitudinal study with retrospective data collection of patients with ADEM seen from 1999 to 2016 at a reference center for demyelinating diseases, identifying demographic, clinical, and laboratory data. Then we compared our findings with data from an extensive review of previously published reports. The literature review was carried out using Google Scholar, PubMed, and the reference lists of included studies. Searches were limited to English language original manuscripts published between 2000 and 2019. RESULTS: Among 1396 registers, we identified 23 cases of ADEM, mostly women (78.3%), Afro-descendant (52.4%) with a mean age of 30.8 ± 11.9 years at onset. One quarter had a previous viral infection and, 4.3% vaccination. The presentation was polyfocal, characterized by the association of pyramidal 82.6%, brainstem 69.6%, mental 65.2%, cerebellar 39.1%, sensory 39.1%, sphincter 43.5%, and visual 34.8% syndromes with severe disability in 86.6%. The breakdown of the blood-brain barrier occurred at 60%. MRI was suggestive of ADEM in 87%, with good radiological evolution. A majority had a significant recovery after treatment. CONCLUSIONS: ADEM in adults is a rare, severe, polyfocal disease with a favorable prognosis. The absence of encephalopathy does not exclude the diagnosis.


Subject(s)
Encephalomyelitis, Acute Disseminated , Adolescent , Adult , Brazil/epidemiology , Child , Encephalomyelitis, Acute Disseminated/diagnostic imaging , Encephalomyelitis, Acute Disseminated/epidemiology , Female , Humans , Longitudinal Studies , Magnetic Resonance Imaging , Retrospective Studies , Young Adult
15.
J Infect Public Health ; 13(7): 1051-1053, 2020 Jul.
Article in English | MEDLINE | ID: mdl-32284196

ABSTRACT

Acute encephalitis is an important cause of mortality and morbidity in children. We retrospectively identified children (≤15 years of age) admitted with suspected encephalitis at the Intensive Care Unit of the Pediatric Department of Cayenne Hospital between January 2007 and December 2018. A total of 30 children with acute encephalitis were identified. The incidence rate varied from 0 to 10.40 cases/100000 children under 15 years. Proven encephalitis was diagnosed in 73% of patients. Nine cases of acute disseminated encephalomyelitis were diagnosed. The causes of infection (44%) were Haemophilus influenzae, followed by Cryptococcus spp and Varicella Zoster Virus. Four children (13%) died: one case of Streptococcus pneumoniae, one of Haemophilus influenzae, one of Mycobacterium tuberculosis and one with no identified cause. Seventeen percent of children had moderate to severe neurological sequelae. The only factor associated with poor outcome was young age at the time of hospitalization (p = 0.03). Conclusion: This study highlights both vaccine-preventable pathogens and acute disseminated encephalomyelitis as the leading causes of childhood encephalitis in French Guiana.


Subject(s)
Encephalitis/epidemiology , Encephalitis/etiology , Adolescent , Age Factors , Child , Child, Preschool , Cryptococcus/pathogenicity , Encephalitis/diagnosis , Encephalitis/microbiology , Encephalomyelitis, Acute Disseminated/complications , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/epidemiology , French Guiana/epidemiology , Haemophilus influenzae/pathogenicity , Herpesvirus 3, Human/pathogenicity , Hospitalization , Humans , Infant , Intensive Care Units , Mycobacterium tuberculosis/pathogenicity , Prognosis , Retrospective Studies , Streptococcus pneumoniae/pathogenicity
16.
Article in English | MEDLINE | ID: mdl-32047077

ABSTRACT

OBJECTIVE: The aims of this study were (1) to describe the incidence of autoimmune encephalitis (AIE) and acute disseminated encephalomyelitis (ADEM) in children, (2) to validate the currently used clinical criteria to diagnose AIE, and (3) to describe pitfalls in the diagnosis of pediatric autoimmune (AI) and inflammatory neurologic disorders. METHODS: This study cohort consists of 3 patient categories: (1) children with antibody-mediated AIE (n = 21), (2) children with ADEM (n = 32), and (3) children with suspicion of an AI etiology of their neurologic symptoms (n = 60). Baseline and follow-up clinical data were used to validate the current guideline to diagnose AIE. In addition, patient files and final diagnoses were reviewed. RESULTS: One-hundred three of the 113 included patients fulfilled the criteria of possible AIE. Twenty-one children had antibody-mediated AIE, of whom 19 had anti-N-methyl-D-aspartate receptor (NMDAR), 1 had anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, and 1 had anti-leucine-rich glioma-inactivated protein 1 encephalitis. Finally, 34 children had ADEM, and 2 children had Hashimoto encephalopathy. Mean incidence rates were 1.54 children/million (95% CI 0.95-2.35) for antibody-mediated AIE and 2.49 children/million (95% CI 1.73-3.48) for ADEM. Of the other 48 children, treating physicians' diagnoses were reviewed. In 22% (n = 6) of children initially diagnosed as having an AI/inflammatory etiology (n = 27), no support for AI/inflammation was found. CONCLUSION: Besides anti-NMDAR encephalitis and ADEM, other AIEs are rare in children. The current guideline to diagnose AIE is also useful in children. However, in children with nonspecific symptoms, it is important to review data critically, to perform complete workup, and to consult specialized neuroinflammatory centers.


Subject(s)
Autoimmune Diseases of the Nervous System/diagnosis , Encephalitis/diagnosis , Practice Guidelines as Topic/standards , Adolescent , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/diagnosis , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/epidemiology , Autoimmune Diseases of the Nervous System/epidemiology , Child , Child, Preschool , Cohort Studies , Encephalitis/epidemiology , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/epidemiology , Female , Humans , Infant , Male , Netherlands/epidemiology
17.
Mult Scler ; 26(7): 806-814, 2020 06.
Article in English | MEDLINE | ID: mdl-31094288

ABSTRACT

OBJECTIVES: The aim of this study was to assess the Dutch nationwide incidence of myelin oligodendrocyte glycoprotein (MOG)-IgG-associated acquired demyelinating syndromes (ADS) and to describe the clinical and serological characteristics of these patients. METHODS: All serum samples for routine diagnostics from February 2014 to December 2017 were sent to the single central reference laboratory for the full-length MOG-IgG cell-based assay (CBA) in the Netherlands. Clinical data from patients known in our National ADS centre were available. RESULTS: A total of 1414 samples of 1277 patients were received; of these, 92 patients (7%) were MOG-IgG-seropositive. The mean incidence was 0.16/100,000 people, with higher seropositivity in children (0.31/100,000) than in adults (0.13/100,000). In MOG-IgG-positive patients at the National ADS centre (61/92, 66%), the most common presenting phenotype is acute disseminated encephalomyelitis (ADEM, 56%) in children and optic neuritis (ON, 44%) in adults. Relapsing disease occurred in 9/34 (26%) children and 11/27 (41%) adults during median follow-up of 27.5 months. Patients were tested MOG-IgG-positive >200 months after the initial attack, suggesting an extended time to first relapse (TTFR). Longitudinal analysis of MOG-IgG (25/61, 41%) showed that 67% of the monophasic patients remain seropositive and 60% in relapsing patients. Majority of seronegative patients had no relapses (89%). CONCLUSION: This nationwide study shows that the overall incidence of MOG-IgG-seropositive disorders is 0.16 per 100,000 people. The distribution over the clinical phenotypes differs between adults and children. Seropositivity can be maintained over years even without clinical activity, while seronegative patients generally had no relapses.


Subject(s)
Demyelinating Autoimmune Diseases, CNS , Myelin-Oligodendrocyte Glycoprotein/immunology , Optic Neuritis , Adolescent , Adult , Autoantibodies/blood , Child , Demyelinating Autoimmune Diseases, CNS/blood , Demyelinating Autoimmune Diseases, CNS/epidemiology , Demyelinating Autoimmune Diseases, CNS/immunology , Demyelinating Autoimmune Diseases, CNS/physiopathology , Encephalomyelitis, Acute Disseminated/blood , Encephalomyelitis, Acute Disseminated/epidemiology , Encephalomyelitis, Acute Disseminated/immunology , Encephalomyelitis, Acute Disseminated/physiopathology , Female , Follow-Up Studies , Humans , Male , Middle Aged , Multiple Sclerosis, Relapsing-Remitting/blood , Multiple Sclerosis, Relapsing-Remitting/epidemiology , Multiple Sclerosis, Relapsing-Remitting/immunology , Multiple Sclerosis, Relapsing-Remitting/physiopathology , Netherlands/epidemiology , Optic Neuritis/blood , Optic Neuritis/epidemiology , Optic Neuritis/immunology , Optic Neuritis/physiopathology , Young Adult
18.
Mult Scler Relat Disord ; 36: 101425, 2019 Nov.
Article in English | MEDLINE | ID: mdl-31655433

ABSTRACT

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) can cause cognitive impairment in children. However, long-term consequences for school performance and psychiatric morbidity have never been characterized. Our aim was to investigate long-term school performance and psychiatric morbidity after pediatric ADEM (<18 years). METHODS: We identified all children with ADEM 2008-2015 in Denmark using hospital diagnostic codes for acquired demyelinating syndromes. We reviewed all medical records to validate ADEM including blinded MRI review. Reference children were the entire pediatric (<18 years) population or randomly sampled sex and age-matched reference children. Outcomes were from nationwide population-based registers on special needs assistance, grade point average, highest completed education, in-hospital psychiatric hospital diagnoses, out-of-hospital psychiatric consultations or psychopharmacological drug prescriptions. RESULTS: 52 children had ADEM (median onset age: 5.5 years; median age at follow-up end: 13.4 years). Secondary school grade point average was similar among children with ADEM and reference children; however, children with ADEM had increased psychiatric morbidity (hazard ratio = 2.4; 95% confidence interval = 1.2-5.1; p = 0.02), primarily due to increased drug prescriptions for sleep problems and depression. CONCLUSION: Children with prior ADEM have increased sleep problems and possibly also depression; however, school performance is seemingly unaffected. Clinicians should consider problems with sleep and mood at follow-up.


Subject(s)
Academic Performance/statistics & numerical data , Encephalomyelitis, Acute Disseminated/epidemiology , Mental Disorders/epidemiology , Registries/statistics & numerical data , Sleep Wake Disorders/epidemiology , Acute Disease , Adolescent , Child , Comorbidity , Denmark/epidemiology , Female , Follow-Up Studies , Humans , Male
19.
Neurosciences (Riyadh) ; 24(2): 89-94, 2019 Apr.
Article in English | MEDLINE | ID: mdl-31056539

ABSTRACT

OBJECTIVE: To evaluate the clinical presenation of acute disseminated Encephalomyelitis (ADEM) in pediatric age group, treatments, and to asses the outcome at King Abdulaziz Medical City, Riyadh, Kingdom of Saudia Arabia. METHODS: The medical records of all patients younger than 18 years of age with a diagnosis of ADEM and treated at King Abdulaziz Medical City from January 1996 to Decemeber 2016 were collected. A total of 20 patients were included. RESULTS: Of 20 patients enrolled in our study, 13 (65%) were female. Autumn and summer were the most common seasons in which ADEM presented (60%); 19 (95%) patients had a history of preceding viral illnesses. The most common neurological deficits on presentation were weakness (85%), ataxia (45%), and nystagmus (45%). Cortical and subcortical lesions (60%) were the most common finding on cranial magnetic resonance imaging. Seventeen patients (85%) received steroid only. Only 16 patients continued with follow-up, with a mean duration of 7 months. All 16 patients improved: 11 patients were recovered and 5 patients still had a neurological deficit at the clinic visits. No patient had relapsed. CONCLUSION: Most of the patients in this case series have an excellent outcome and attended follow-up visits and no disease relapses were identified. Further exploration of the disease is recommended.


Subject(s)
Encephalomyelitis, Acute Disseminated/epidemiology , Adolescent , Brain/diagnostic imaging , Brain/pathology , Child , Child, Preschool , Encephalomyelitis, Acute Disseminated/diagnosis , Encephalomyelitis, Acute Disseminated/pathology , Female , Humans , Infant , Male , Retrospective Studies , Saudi Arabia/epidemiology , Seasons , Tertiary Care Centers/statistics & numerical data , Tertiary Healthcare/statistics & numerical data
20.
Mult Scler Relat Disord ; 32: 41-45, 2019 Jul.
Article in English | MEDLINE | ID: mdl-31030018

ABSTRACT

OBJECTIVE: To clarify the features of callosal lesions on magnetic resonance imaging (MRI) in Multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and acute disseminated encephalomyelitis (ADEM). METHODS: Chinese patients diagnosed with MS (n = 33), NMOSD (n = 31), and ADEM (n = 18) were enrolled. Characteristics of lesions in corpus callosum were evaluated with 1.5 Tesla MRI scanners. Chi-squared test (Fisher's exact test) was used to analyze the data. RESULTS: In corpus callosum, NMOSD and ADEM lesions tend to have a diffuse distribution (p = 0.006, p = 0.033) and blurred margins (p < 0.001, p = 0.017), when compared with MS; lesions in NMOSD were less ovoid (p = 0.006), while fewer lesions in ADEM existed in the rostrum and genu (p = 0.002). NMOSD has the most heterogeneous intensity on post-enhancement sequences (p = 0.016, p = 0.001). Radial-like lesions were more common in MS and NMOSD (p = 0.019, p < 0.001). CONCLUSION: MS lesions were most likely focally-localized with clear margins. Radial callosal lesions are characteristic of MS and NMOSD but rarely seen in ADEM. The signal intensities of the lesion were the most heterogeneous in NMOSD. Therefore, the lesion patterns in corpus callosum may serve as a useful clue for correct diagnosis, facilitating early treatment.


Subject(s)
Corpus Callosum/diagnostic imaging , Encephalomyelitis, Acute Disseminated/diagnostic imaging , Magnetic Resonance Imaging , Multiple Sclerosis/diagnostic imaging , Neuromyelitis Optica/diagnostic imaging , Adult , China/epidemiology , Encephalomyelitis, Acute Disseminated/epidemiology , Female , Humans , Magnetic Resonance Imaging/methods , Male , Multiple Sclerosis/epidemiology , Neuromyelitis Optica/epidemiology , Retrospective Studies
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