Acute Disseminated Encephalomyelitis In Children Research Articles

Epidemiological and clinical characteristics of myelin oligodendrocyte glycoprotein antibody-associated disease in a nationwide survey

Background: To our knowledge, no nationwide epidemiological study of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has been conducted. Objective: We examined the epidemiology and clinical features of MOGAD in Japan. Methods: We distributed questionnaires on the clinical characteristics of patients with MOGAD to neurology, pediatric-neurology, and neuro-ophthalmology facilities throughout Japan. Results: In total, 887 patients were identified. The estimated number of total and newly diagnosed MOGAD patients was 1,695 [95% confidence interval (CI): 1483–1907] and 487 (95% CI: 414–560), respectively. The estimated prevalence and incidence were 1.34/100,000 (95% CI: 1.18–1.51) and 0.39/100,000 (95% CI: 0.32–0.44), respectively. The median age at onset was 28 years (range: 0–84 years). At onset, optic neuritis was present in approximately 40% of patients, irrespective of the onset age. Acute disseminated encephalomyelitis was more frequent in younger patients, whereas brainstem encephalitis, encephalitis, and myelitis were more frequent in elderly patients. Immunotherapy was highly effective. Conclusion: The prevalence and incidence rates of MOGAD in Japan are similar to those in other countries. Notable characteristics such as the preferential occurrence of acute disseminated encephalomyelitis in children exist; however, general characteristics including symptoms and treatment response are common irrespective of the onset age.

Diagnostic odyssey of acute disseminated encephalomyelitis in children

We aimed to determine whether acute disseminated encephalomyelitis (ADEM) diagnosis in children is delayed, and if so, to identify the clinical risk factors of delayed diagnosis. Standardised data were collected from children with ADEM from 2003 to 2020. Overall diagnostic delay (time between symptom onset and ADEM diagnosis), physicians’ delay (between the first medical visit and ADEM diagnosis), and patients’ delay (between symptom onset and the first medical visit) were analysed. Thirty ADEM patients were identified, including 16 (54%) with neurological deficits at discharge. Overall, physicians’, and patients’ delays were 9 (interquartile range [IQR] 6–20.5), 5.5 (IQR 3–14), and 4 (IQR 2–8) days, respectively. Overall delay was significantly associated with physicians’ delay, but not with patients’ delay. There were 61 misdiagnoses among 25 (83%) patients, while 5 (17%) were diagnosed correctly at the first visit. The misdiagnoses of common respiratory and gastrointestinal infection and aseptic meningitis were associated with overall and/or physicians’ delay. Later onset of specific neurological features suggestive of ADEM was associated with all three diagnostic delays. A unique diagnostic odyssey exists in ADEM. Several clinical risk factors were associated with the diagnostic delay.

A study on acute disseminated encephalomyelitis in children

A study on acute disseminated encephalomyelitis in children

Nursing Interventions of Children with Acute Disseminated Encephalomyelitis

This paper aims to investigate the clinical features and nursing interventions of children with acute disseminated encephalomyelitis (ADEM). By randomly selecting the children with ADEM treated in our hospital as the study subjects, the relevant clinical data of these patients was analysed and then their clinical characteristics were compared. According to the randomized table method, the patients were divided into control group and study group, where the control group was given routine nursing care, while the study group was given comprehensive care, so as to compare and analyse the effects of the two groups. The results show that the incidence of ADEM in children has no significant difference in the four seasons, and the predisposing factors are related to respiratory tract infection, viral infection, gastrointestinal tract infection and vaccination. In terms of clinical typing, it includes brain type and cerebrospinal type. The clinical manifestations of ADEM are fever, limb spasms, and convulsions. Therefore, the clinical symptoms of ADEM are rather clear, and the timely symptomatic treatment and assisted effective nursing intervention can improve the therapeutic effect.

Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination

ObjectiveWe characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination.MethodsWe evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy...

Validated age-specific reference values for CSF total protein levels in children.

To define age-specific reference values for cerebrospinal fluid (CSF) total protein levels for children and validate these values in children with Guillain-Barré syndrome (GBS), acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS). Reference values for CSF total protein levels were determined in an extensive cohort of diagnostic samples from children (<18 year) evaluated at Erasmus Medical Center/Sophia Children's Hospital. These reference values were confirmed in children diagnosed with disorders unrelated to raised CSF total protein level and validated in children with GBS, ADEM and MS. The test results of 6145 diagnostic CSF samples from 3623 children were used to define reference values. The reference values based on the upper limit of the 95% CI (i.e. upper limit of normal) were for 6 months-2 years 0.25g/L, 2-6 years 0.25g/L, 6-12 years 0.28g/L, 12-18 years 0.34g/L. These reference values were confirmed in a subgroup of 378 children diagnosed with disorders that are not typically associated with increased CSF total protein. In addition, the CSF total protein levels in these children in the first 6 months after birth were highly variable (median 0.47g/L, IQR 0.26-0.65). According to these new reference values, CSF total protein level was elevated in 85% of children with GBS, 66% with ADEM and 23% with MS. More accurate age-specific reference values for CSF total protein levels in children were determined. These new reference values are more sensitive than currently used values for diagnosing GBS and ADEM in children.

NEUROPHYSIOLOGY PARAMETERS IN DIAGNOSTICS OF MULTIPLE SCLEROSIS AND ACUTE DISSEMINATED ENCEPHALOMYELITIS IN CHILDREN

Our research objective was to evaluate the importance of neurophysiological methods in diagnosing the state of visual, somatosensory and motor pathways condition in the early stages of multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM) in children. Materials and methods. Twenty-four children with a debut of multiple sclerosis, 15 children with debute of acute disseminated encephalomyelitis and 20 neurologically healthy children of the comparison group were examined. All patients were evaluated by neurologist, brain MRI and CSF analysis (isoelectrofocusing to oligoclonal IgG, oligoclonal bands test), visual evoked potentials (VEP), transcranial magnetic stimulation (TMS) and somatosensory evoked potentials (SSEP). Results. In children with MS asymmetry of the conduction along the motor pathways on the spinal level was higher than in patients with ADEM and controls, functional state of somatosensory cortex neurons was lower and conduction along somatosensory pathways on the spinal level was slower – all differences significant. According to the visual evoked potentials, in more than half of the cases, there was an increase in the latency of the P100 peak. Also in MS group there was a significant disruption of the visual pathway in 54% of the cases. Neurophysiological changes in 58% of cases were demyelinating, and violations of the axonal type occurred in 37% of cases. Conclusions. Neurophysiological diagnostic methods such as transcranial magnetic stimulation, visual evoked potentials, somatosensory evoked potentials are highly informative for the differential diagnosis of multiple sclerosis and acute disseminated encephalomyelitis. More pronounced spinal lesions in early stages of MS than in ADEM in children may be the cause of the neurophysiologic differences, and prevalence of the sensory system involvement at this stage may be the reason behind more extended SSEP abnormalities comparing with TMS. VEP changes may reflect primary demyelinating course of the disease on the early stages of MS. VEP, SSEP and TMS may be recommended as standard way of evaluation and diagnostic of ADEM and MS in children.

Pattern and Outcome of Acute Disseminated Encephalomyelitis (ADEM) in Children: Experience in a Tertiary Center, Upper Egypt.

IntroductionAcute disseminated encephalomyelitis (ADEM) is an immune mediated disease of the brain. Although it occurs in all ages, most reported cases are in children and adolescents. The aims of this study were to study the clinical pattern and outcome of ADEM in children in a tertiary center in Upper Egypt and to determine the effect of combined use of steroids and IVIg on outcome.MethodsThis observational study was carried out from January 2014 through December 2014 in the Pediatric Department of Sohag University Hospital (Egypt). All children diagnosed as ADEM during a one year period were included in this study. The treatments used were IV methylprednisolone followed by oral prednisone taper and intravenous immunoglobulin for severe cases. All studied cases were followed up and reevaluated at three months and six months. We used SPSS version 10 and Chi Square, Spearman’s test and t-test for data analysis.ResultsEighteen children were included in this study (10 males and 8 females), the average age was 5.5 ± 0.9 years. Prodroma was found in 72.22% of the cases while the main complaint was encephalopathy (83.33%) followed by seizures (11.11%). The neurological findings were convulsions in 83.33%, quadriparesis (33.33%), hemiparesis (33.33), bladder involvement (both retention and incontinence) in 61.11%, and cranial nerve affection (11.11%). Demyelination patches were multifocal in 50%, mainly subcortical in 27.78%. Intelligence quotient (IQ) assessment after 6 months follow up showed that 50% were below average, 25% had mild MR while neurological evaluation showed that 75% of our patients were completely cured. The predictors of better outcome were; children related to the age group (1–4 years) (p = 0.01), children with higher GCS (6–14) (p = 0.01), and children who received steroids on the first day of symptoms and intravenous immunoglobulin in the first week (p = 0.03).ConclusionThe clinical pattern of acute disseminated encephalomyelitis is variable, and a disturbed level of consciousness was the most common presentation. The outcome is generally favorable although motor deficit and cognitive impairment were reported. The combined use of steroids and IVIg has substantial effect on the outcome in children with ADEM.

Multimodal treatment of acute disseminated encephalomyelitis in children

The chief treatment options for acute disseminated encephalomyelitis (ADEM) include methylprednisolone (MP), plasma exchange (PE), intravenous immunoglobulin (IVIG). However, there is no evidence-based recommendations for management of ADEM. To identify the prognosis in ADEM after use of different modalities of treatment, a 3-year prospective study of cases presenting with neurological features with a temporal relation to an infection or vaccination or with a presumed etiology as demyelination was undertaken. Investigations to identify the causative agent, magnetic resonance imaging of brain and spinal cord, cerebrospinal fluid studies, electrophysiological studies, blood tests to exclude metabolic and collagen vascular disorders were done. A standard protocol of steroids, failing which (Modified Rankin scale score of four or 5 at end of 3 weeks) IVIG or PE was given. One patient underwent hemicraniectomy. Cases were followed up for 1 year. Telephonic interview was done at 3rd and 5th year. Of the 32 cases (

Severe Acute Disseminated Encephalomyelitis Following Swine Influenza Virus (H1N1) Infection in Children

Background: Neurological complications of influenza viral infection have rarely been discussed in current literature. In the era of recent outbreaks of this potentially serious viral illness, understanding of possible extrapulmonary clinical manifestations is crucially needed. Case report: We are reporting two toddler girls who have been endorsed as previously healthy. Both patients have presented with a febrile respiratory illness followed by severe encephalopathy needing intensive care unit admission. They have been found to have novel H1N1 infection. Their neuroimaging studies were consistent with severe acute disseminated encephalomyelitis (ADEM). Good response to immunotherapy has been luckily achieved. Discussion: ADEM is an acute autoimmune encephalopathy disease which is commonly triggered by viral illnesses. However, particular correlation with novel H1N1 viral infection is not well described. Whether H1N1 triggers more severe clinical and radiological variant of ADEM is not yet precisely known, though has been observed in our study. Current knowledge in regard to neurological burden of H1N1 infection is reviewed. Conclusion: It is likely that H1N1 can trigger a more severe form of ADEM in children. Early suspicion and isolation of H1N1 virus in children who present with acute unexplained encephalopathy in the context of any viral illness seems to be a recommended practice.

Acute Disseminated Encephalomyelitis in Children and Adolescents: A Single Center Experience

BackgroundAcute disseminated encephalomyelitis is an immune-mediated disease that produces multiple inflammatory lesions in the brain and spinal cord.MethodsThis study retrospectively evaluated 15 children with acute disseminated encephalomyelitis in children and adolescents from a single institution in Adana, Turkey.ResultsThe patients presented in a seasonal distribution, with 73.3%: (11/15) presenting in winter or spring. The majority of patients (13/15, 86.7%) had a history of acute febrile illness 2 to 40 days before presentation, and five children had serologic evidence of specific triggers: mycoplasma (2 children), influenza-A (H1N1) (1 child), or Epstein-Barr virus. All children were treated with a standard protocol of 3 to 5 days of intravenous administration of methylprednisolone and intravenous immunoglobulin for patients who continued to deteriorate. Oseltamivir and clarithromycin were administered in patients with influenza-A (H1N1) and mycoplasma according to the serology. In 13 patients, all neurologic signs and symptoms resolved after treatment. Only one patient was left with severe neurologic sequelae and another child had recurrent attacks and was ultimately diagnosed with possible multiple sclerosis.ConclusionsThe present series demonstrates that acute disseminated encephalomyelitis in children occurs predominantly in winter or spring and often follows an upper respiratory tract illness for those along the southern coast of Anatolia (Mediterranean region). Early treatment with immunomodulative agents is recommended and is likely to result in a favorable outcome or full recovery. This study also suggests benefit from antiviral and antibiotic treatment initiated as soon as possible after the onset of illness.

The Magnetic Resonance Imaging Appearance of Monophasic Acute Disseminated Encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is an immunologically mediated inflammatory disease of the central nervous system that typically occurs after a viral infection or recent vaccination, and is most commonly seen in the pediatric population. In 2007 the International Pediatric Multiple Sclerosis Study Group proposed a consensus definition for ADEM for application in research and clinical settings. This article gives an overview of ADEM in children, focusing on differences that have emerged since the consensus definition was established. Although the focus is on neuroimaging in these patients, a synopsis of the clinical features, immunopathogenesis, treatment, and prognosis of ADEM is provided.

Can “dissemination” include the PNS in adults with acute disseminated encephalomyelitis?

Acute, noninfectious, inflammatory illnesses of the CNS encompass an increasingly diverse array of clinical and biological entities, including acute disseminated encephalomyelitis (ADEM), brainstem encephalitis, and paraneoplastic and non-neoplastic antibody–associated encephalopathies. ADEM is classically a monophasic, inflammatory-mediated attack on the CNS, more frequently seen in children than adults.1 According to international consensus criteria, ADEM in children is clinically defined by encephalopathy, polyfocal neurologic deficits, and typified by ill-defined multifocal lesions of the brain and potentially of the optic nerves and spinal cord.2 In ADEM, a history of a prodromal illness in the days before the onset of neurologic deficits implicates microbial exposure as an inciting contribution to the subsequent host immune response. No single infectious agent has been causally linked to ADEM, however, and no reliable biomarker defines this illness. As such, ADEM may encompass either a spectrum of disorders or a spectrum of clinical phenotypes that share some common aspects of pathobiology. Refinements within the clinical continuum of acute inflammatory illnesses of the CNS are necessary when the existence of discernible subgroups compels the consideration of important implications for acute therapy and prognosis. In this issue of Neurology ® , Marchioni et al.3 have made such a refinement by identifying a not uncommon subgroup of postinfectious ADEM in adults, which has mixed central and peripheral nervous system (PNS) involvement.

Çocukluk çağında akut dissemine ensefalomiyelit: Tek merkez deneyimi

Objective: To describe the epidemiologic, clinical, neuroimaging, and laboratory features; treatment; and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM). Methods: Clinical features, laboratory and radiological findings of 14 patients with the diagnosis of ADEM were recorded in 2004-2010 in the range of 6-year follow-up time period in Pediatric Neurology Department of Selcuk University. Results: Ten patients were boys and four were girls. Ten patients presented either in winter or in spring. The majority of patients (64.2%) had as history of acute febrile illness. Patients presented most often with gait disturbances (71.4%) and secondly with altered consciousness (57%). Despite rigorous microbiologic testing, a definite microbiologic diagnosis was established only in four patients. Brain magnetic resonance imaging (MRI) identified lesions in the cortex and, or in subcortical white matter, especially in the frontal (57%) and temporal (42%) regions. Ten patients were treated with corticosteroids, and four were treated with intravenous immunoglobulin. Ganciclovir and clarithromycin were administered in patients with Epstein-Barr virus and mycoplasma according to the serology. In 13 patients, all neurologic signs and symptoms resolved after treatment. Conclusion: The present series demonstrates that acute disseminated encephalomyelitis in children occurs predominantly in winter and spring and often follows an upper respiratory tract illness.

Acute Disseminated Encephalomyelitis: A Long-Term Prospective Study and Meta-Analysis

There are only a few series in the literature on acute disseminated encephalomyelitis (ADEM) in children. the aims of this study were to perform (i) a prospective clinical/imaging study (1992-2009) on ADEM in children consecutively referred to our institution in Catania, Italy, and (ii) to undertake a systematic review and meta-analysis of published ADEM pediatric cohorts (>10 cases). We identified 17 patients with ADEM (incidence <10 years of age=1.1 per 100 000 person-years). 15 previously published cohorts were compared with our cohort: (i) systematically reviewed (750 cases); and (ii) meta-analyzed (492/750 cases). The 17 patients had the following characteristics: (a) male-to-female ratio, 1.4 (vs. 1.2-1.3 in previous cohorts); (b) mean age at presentation, 3.6 years (vs. 7.1 years in previous cohorts); (c) specific preceding triggering factor, 88% (vs. 69-79% in previous cohorts); (d) the most common initial signs were ataxia, seizures, headache, and thalamic syndrome; (e) brain imaging revealed >3 lesions in 100% (vs. 92% in previous cohorts); (f) the outcome was good in 94% (vs. 70-75% in previous cohorts); and (g) 12% relapsed once (vs. 18% in previous cohorts). ADEM is generally a benign condition that mosly affects boys more than girls and rarely recurs.

Clinical profile of acute disseminated encephalomyelitis in children

Aim:To study the clinical profile of acute disseminated encephalomyelitis (ADEM) in children.Materials and Methods:All children admitted with ADEM during a period of one and a half years were included in the study. The diagnosis of ADEM was made based on the clinical presentation and suggestive MRI findings. All children were treated with intravenous methyl prednisolone, followed by oral prednisolone and followed up for varying periods up to three and a half years.Results:The sample consisted of 14 children with 11(79%) girls and 3 (21%) boys. The oldest child was 12 years and the youngest was a six-month-old infant. Acute febrile illness preceded the onset of neurological symptoms in 64% of children. The interval between the preceding illness and symptoms of ADEM varied from 7 days to 28 days (mean 12 days). The common presenting symptoms were fever, vomiting, headache, gait disturbance and generalized seizures. Neurological manifestations included altered sensorium, multiple cranial nerve involvement, quadriplegia and paraplegia, dystonia and choreiform movements, nystagmus, bladder involvement (both incontinence and retention), speech defect and double vision. Facial nerve was the most common cranial nerve involved. Psychological manifestations included aggressive behavior, psychotic symptoms and mood changes. One child each had features of acute psychotic episode and depressive episode. All children recovered fully. One child had multiphasic disseminated encephalomyelitis (MDEM) on follow up.Conclusion:Despite the serious neuropsychiatric manifestations, ADEM in children generally has good immediate outcome. Children with ADEM need long-term follow up for cognitive impairments.

Sorting through the pediatric MS spectrum with brain MRI

Distinguishing between a first episode of multiple sclerosis and acute disseminated encephalomyelitis in children who present with an initial demyelinating event can be a clinical challenge. New brain MRI criteria that aim to differentiate these clinical presentations, and revised McDonald MRI criteria specific for the pediatric population, are both worthy of note.

Acute disseminated encephalomyelitis cohort study: Prognostic factors for relapse

To date, there is no available epidemiological study about prognostic factors of acute disseminated encephalomyelitis (ADEM) in children, using a cohort of patients with homogenous inclusion criteria. We aimed to evaluate prognostic factors for relapse after ADEM in children. A total of 132 children from the French National KIDSEP Neuropediatric Cohort (mean age at onset: 6±3.3 years; mean follow-up: 5.4±3.3 years; lost to follow-up: 10%). ADEM diagnosis was considered in a previously healthy patient acutely presenting more than one neurological deficit, change in mental state and MRI alterations including white matter changes. We used multivariate survival analysis (Cox model) evaluating the prognostic value of baseline clinical, biological and MRI covariates, for the occurrence of a second attack. Twenty-four (18%) of included patients had a second attack. An increased risk of relapse was associated with optic neuritis (hazard ratio, 5.23; 95% CI, 2–13.65), familial history of central nervous system inflammatory demyelination (7.79; 1.54–39.5), Barkhof multiple sclerosis (MS) criteria on MRI (2.52; 1.04–6.12) and no neurological sequelae after first attack (3.79; 1.12–12.85). Clinical and MRI prognostic factors for relapse in ADEM may contribute to an early distinction between monophasic and relapsing disease, which may be related to MS.

Acute Disseminated Encephalomyelitis in North Indian Children: Clinical Profile and Follow-Up

Acute disseminated encephalomyelitis in children is not uncommon in developing countries, yet there is little systematic documentation of its clinical profile and follow-up. We studied the clinical and neuroradiologic features of acute disseminated encephalomyelitis in 52 consecutive children. Clinical details, magnetic resonance imaging (MRI) findings, and the results of other investigations were recorded, and children were followed up from 6 to 48 months. A repeat MRI was done after 3 to 4 months, and in those with persistent lesions, another MRI was done after 6 to 7 months of discharge. The mean age at presentation was 6.14 +/- 3.17 years, 73.1% were male, and 17 children had a history of antecedent infectious illness or vaccination. Most children had a meningoencephalitic presentation, with sudden-onset motor weakness in 76.9% and seizures in 36.5%. Altered sensorium and pyramidal signs were seen in 55.8% and 80.7% of children, respectively. On MRI, scattered T(2)-weighted hyperintense lesions were seen, mainly in the subcortical white matter, especially in the parietal (53.8%) and frontal (30.17%) regions. Thalamic, basal ganglia, and callosal lesions were seen in 30.76%, 17.3%, and 13.46% of cases, respectively. Variable contrast enhancement was seen in 48% of those who had contrast MRIs. The response to methylprednisolone was good, with dramatic recovery in 26.9% and marked improvement in 51.9% at discharge. On follow-up, of 44 children, residual smaller MRI lesions were seen in 30. The MRI was repeated at 6 months in children with residual lesions, and it was found that the lesions either disappeared or were significantly reduced after 6 months in 75% of cases. Four children had relapse of acute disseminated encephalomyelitis with new lesions on MRI. All of them responded to methylprednisolone. None of the clinical or neuroradiologic factors at presentation had any significant correlation with relapse. Six months after discharge, no deficits could be found in 61.3% of cases; 15.9% and 4.5% had motor and cognitive deficits, and 9% had multiple deficits. The presentation of pediatric acute disseminated encephalomyelitis in developing countries is similar to that in developed countries. In spite of an aggressive presentation, most children respond well to corticosteroids. MRI lesions disappear or are significantly reduced at 6 months in the majority of cases.

Acute disseminated encephalomyelitis in children: one medical center experience.

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disease of the central nervous system. The experience in children is limited. We retrospectively reviewed our experience with 20 ADEM patients (10 females, 10 males) with age of onset before 18 years old in Taiwan to clarify the clinical manifestations, neuroimaging findings, and the relationship between ADEM and multiple sclerosis (MS). The age at onset ranged from 4 months to 15 years. Seventeen (85%) children had a recent infectious prodrome. Children presented most often with acute consciousness disturbance (70%) and motor deficits (55%). Seizures occurred in 10 (50%), but only one child developed epilepsy in follow-up. Brain magnetic resonance imaging (MRI) evaluations done in all patients revealed multifocal lesions, mainly in subcortical white matter (80%), brainstem (65%), basal ganglia (55%), cerebellum (45%), thalamus (40%), and periventricular white matter (35%). Spinal cord MRI was performed in 9 patients and all of them showed abnormal lesions. Eleven patients were treated with high-dose intravenous methylprednisolone pulse therapy, and only one had mild long-term neurological sequelae. Among the 20 patients, five had long-term neurological sequelae and one died. Three patients fit the criteria of multiphasic disseminated encephalomyelitis, in which two developed MS in follow-up. Another patient with ADEM turned out to be MS two years later. We concluded that seizures are not uncommon in ADEM, but the subsequent development of epilepsy is rare. Long-term prognosis of ADEM is generally good. Because recurrence of ADEM is not uncommon, long-term follow-up of those children with ADEM is needed to distinguish between ADEM and MS.