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.

The Wide Variety of Acute Disseminated Encephalomyelitis in Children: A Clinical Perspective

Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating inflammatory disorder of the central nervous system. It is characterized by encephalopathy, multifocal neurological deficits, and typical magnetic resonance imaging findings of widespread demyelinating lesions, predominantly involving the white matter of the brain and spinal cord. ADEM mainly affects children and is commonly associated with preceding viral and bacterial infections, and, rarely, vaccinations. Despite substantial advances in the understanding of the association of myelin oligodendrocyte glycoprotein antibody with recurrent forms of ADEM or other demyelinating conditions, specific etiologic agents or biological markers have not been identified. Therefore, the diagnosis of ADEM is still based on clinical and radiological findings and the exclusion of other conditions mimicking ADEM. However, a prompt diagnosis and adequate treatment are crucial because diagnostic delays or inappropriate treatment may lead to unwanted neurological sequelae in some children. There is no standardized treatment protocol for ADEM, but the use of corticosteroids, intravenous immunoglobulin, and plasmapheresis has been associated with good clinical outcomes. Adequate treatment has reportedly resulted in favorable outcomes, with full or almost full recovery in most children with ADEM, although some children may develop neurological sequelae, such as cognitive impairment and motor deficits. Further studies are needed to identify biological clues and optimal treatment protocols to minimize the incidence of neurological sequelae.

Acute disseminated encephalomyelitis in children and adolescents - 20-year single-center experience in Serbia

Introduction/Objective. Acute disseminated encephalomyelitis (ADEM) is the most common demyelinating disease of the central nervous system in pediatric patients. We aimed to evaluate the clinical profile of children with ADEM and to discern prognostic factors for disease outcome. Methods. A 20-year retrospective?prospective study was conducted in a cohort with the diagnosis of ADEM. Results. The study included 36 patients, with range of follow-up period of 6?120 months (median of 26 months). Prior infection was reported in 72.2% of the patients. In the clinical presentation of the disease, motor deficit was most common (81.1%), followed by ataxia (77.8%). More than a third of patients had back and limb pain or abdominal visceral pain, which highly correlated with MRI findings of myelitis. Abnormal brain CT findings were evident in 22.2% of the patients, and this was associated with higher Expanded Disability Status Scale (EDSS) and quicker progression of the disease. Median EDSS was 0 at the most recent follow-up visit, in all the patients. EDSS 0?2.5 was verified in 29 (80.6%) of the patients, while three (8.3%) patients scored 7?9.5 at the last visit. Two patients had a lethal outcome. Conclusions. ADEM is a serious disease in pediatric patients, but with a good prognosis, which is illustrated by the fact that 80.6% of our patients had a complete or almost complete recovery.

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.

Update on Acute Disseminated Encephalomyelitis in Children and Adolescents.

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory demyelinating disease of the central nervous system (CNS) that usually affects children and young adults after an infection or vaccination. The presence of several conditions mimicking ADEM, added to the lack of specific biomarkers, makes diagnosis potentially hard. Prompt diagnosis is necessary to start adequate treatment to improve the clinical course and long-term outcome. Because of its heterogeneity in both clinical presentation and course, challenges remain in establishing the most appropriate therapeutic approach in each patient. The aim of this review is to provide an update on management of this disease with a focus on acute treatment and to give suggestions for future research. We showed that there are currently no guidelines that help clinicians manage ADEM and therapeutic decisions are often made on a case-by-case basis. Further studies are necessary to identify clinical, laboratory, and instrumental criteria that could be correlated with outcomes and guide clinicians in choosing when and what treatment should be given in each case.

Clinical Profile of Acute Disseminated Encephalomyelitis in Children- An Experience from a Tertiary Care Centre in Eastern India

Clinical Profile of Acute Disseminated Encephalomyelitis in Children- An Experience from a Tertiary Care Centre in Eastern India

A study on acute disseminated encephalomyelitis in children

A study on acute disseminated encephalomyelitis in children

Acute disseminated encephalomyelitis in children - clinical and MRI decision making in the emergency department

BackgroundAcute disseminated encephalomyelitis (ADEM) is an uncommon, treatable, primarily pediatric, immune-mediated disease. Diagnosis of ADEM requires two essential elements: typical clinical presentation and magnetic resonance imaging (MRI) findings. The aim of this study was to evaluate how clinical findings in the initial emergency department (ED) presentation influenced the timing of MRI. MethodsA retrospective chart review was conducted of children diagnosed with ADEM, over a 12-year period, in a tertiary care pediatric center. Clinical presentation at ED admission was recorded and patients who underwent an MRI as part of their ED evaluation (early MRI) with those who had MRI performed during ward hospitalization (late MRI) were compared. Results30 patients were diagnosed with ADEM during the study period. Encephalopathy and polyfocal neurological signs were described in 80% and 50% of patients ED charts, respectively. Seven patients underwent early MRI and polyfocal neurological signs were more common in this group (p = 0.006). Fever was more common in the late MRI group (p = 0.02). Following diagnosis, all patients were treated with immune-modulation therapy, improved clinically, and were discharged. Conclusion20% of ADEM patients were not encephalopathic at ED presentation. Polyfocal neurological signs and absence of fever at ED presentation were related to earlier MRI utilization and thus earlier diagnosis and treatment. Familiarity with the ADEM constellation of signs, and a high index of suspicion, may help the ED clinician in early diagnosis and treatment of this rare disease.

Epidemiological, Clinical, and Laboratory Characteristics of Acute Disseminated Encephalomyelitis in Children: A Retrospective Study.

We aimed to study the precipitating factors, demographic data, clinical and radiological manifestations, electroencephalography and laboratory findings, as well as association with infections, immunization and incidence of relapse of acute disseminated encephalomyelitis (ADEM) in children admitted to Mofid Children Hospital, Tehran, Iran from Mar 2013 to Mar 2016. A 3-yr retrospective review of 29 children with definite final diagnosis of ADEM in Mofid Hospital in Tehran, Iran was performed. The diagnosis was based on specified criteria, including a presumed acute demyelinating process with no history of unexplained neurological symptoms and at least one demyelinating lesion shown on magnetic resonance imaging without evidence of previous destructive white matter lesions. Overall, 29 children diagnosed as ADEM were studied in terms of demographic characteristics, clinical manifestations and laboratory findings in two groups according to their recurrence. The mean age of the patients with recurrence was less than those without it were. It was more common in females but the difference was not statistically meaningful. There was no relationship between the season of the first episode of the disease and the recurrence incidence. Moreover, the relationship between viral infections and recurrence was statistically non-meaningful. No relationship between the recurrence of ADEM and clinical manifestations, radiological and laboratory findings was found. The reason for high rate of recurrence in our patients may be related to the younger age of children in our study.

Cytoflavin efficacy in the treatment of disseminated encephalomyelitis in children

To evaluate the efficacy of cytoflavin in the complex therapy of disseminated encephalomyelitis in children. A blind, placebo - controlled, randomized study was carried out. Thirty-two patients, aged form 1 to 17 years, received cytoflavin from the first day after admission in intravenous drips in 5% glucose solution during 10 days. The control group consisted of 30 children who received a standard infusion therapy. Clinical and laboratory evaluation was carried out on the 11th and 30th days, MRT was performed before hospitalization and on the 30th day. Cytoflavin had a positive effect on the rate of recovery of the majority of neurological symptoms. The average EDSS score decreased by 1.5 times to the 30th day compared with the control group. The complete regression of clinical symptoms and focal changes on MRI on the 30th day was observed in 25% of children of the main group and in 3.3% in the control group (р=0.001). The drug exerted the endothelium protective and antioxidant effects, improved blood rheology and restored the content of circulating endothelial cells, the level of D-dimer and indicators of free radical oxidation to the 30th day of treatment.

Plasma Exchange for Autoimmune Encephalitis and Acute Demyelinating Encephalomyelitis in Children: Clinical and Radiological Characteristics

Autoimmune encephalitis (AE) is a term used to indicate onset of acute neuropsychiatric symptoms with deterioration of consciousness, seizures and focal neurological deficit secondary to a presumed autoimmune aetiology. Infections such as acute bacterial or viral encephalitis, toxins and other causes of encephalopathy should be excluded. Additionally, a search for an aetiological factor should include testing blood and CSF for the likely causative antibodies.

Pathologic Studies of Fatal Encephalomyelitis in Children Caused by Enterovirus 71.

Enterovirus 71 (EV71) is the major pathogen of hand, foot, and mouth disease and can cause death; however, its pathogenesis remains elusive. We performed a detailed systematic histopathologic examination and molecular studies on six autopsy cases of EV71 infection using H&E, immunohistochemistry, double immunofluorescence staining, and nested reverse transcription polymerase chain reaction. Characteristic features of acute encephalomyelitis were observed. Viral antigens were mainly detected in neuronal cytoplasm and processes in the different brainstem nuclei and spinal cord, including the anterior and posterior horn cells. Viral antigens were also positive in the nerve roots of spinal cord and autonomic ganglia of intestines. Our study revealed direct pathologic evidence supporting viral entry into the central nervous system (CNS) through peripheral nerves. In addition to the major motor pathway, EV71 can also enter the CNS via peripheral sensory and autonomic pathways in retrograde axonal transport.

Acute disseminated encephalomyelitis in children.

Acute disseminated encephalomyelitis in children.

Acute disseminated encephalomyelitis in children: Characteristics of cases in Jordan

Our purpose is to describe the clinical, neuroradiologic features and outcome of patients with acute encephalomyelitis (ADEM) from Jordan, a developing country, and to compare the findings to other reports from the developed and developing countries. A retrospective review was conducted of the medical records and magnetic resonance imaging (MRI) findings of children who presented to any of the three hospitals that were involved in the study in Jordan from 2002 to 2005. Eleven children were diagnosed with ADEM by clinical and MRI findings. All were treated with steroids and evaluated at 6–24 month follow-up. Three of the children (27% presented with a history of recent upper respiratory tract infection, but none presented with a recent history of vaccination. Prodromal signs and symptoms were reported in only six (55% of the children. All the children except one had a motor deficit. Only three (27% of the children had abnormal cerebrospinal fluid (CSF) findings and eight had normal results. MRI lesions were most commonly bilateral and asymmetric but varied in shape and size. The use of methylprednisolone was usually associated with reasonable recovery. Nine patients (82%) recovered completely while two children recovered with sequelae. Compared to reports from developed countries, this series has a lower prevalence of preceding non-specific infection and a higher prevalence of normal CSF findings. These findings may be related to a difference in etiologic pathogens. Therefore, a larger regional multicenter study is highly recommended.

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 (

Acute disseminated encephalomyelitis in children

L'encéphalomyélite aiguë disséminée (ADEM) est une maladie inflammatoire, démyélinisante, multifocale intéressant principalement la substance blanche du système nerveux central. Elle est rare mais non exceptionnelle chez l'enfant. Les auteurs rapportent une étude colligeant 9 cas d'ADEM pris en charge au service de Pédiatrie du CHU Hassan II à Fès, sur une période de 4 ans. Il s'agit de cinq garçons et quatre filles; âgés entre 2 ans et 13 ans. Les antécédents (ATCD) d'infection virale sont notés chez quatre patients. La fièvre est constante. Les convulsions sont présentes dans cinq cas et les troubles de conscience dans cinq cas. L'examen neurologique était anormal. L’étude de liquide céphalo-rachidien a révélé une réaction méningée. On ne notait pas de syndrome inflammatoire biologique. L'imagerie par résonance magnétique (IRM) cérébrale a objectivé des lésions en hyper-signal en T2 et T2 FLAIR chez tous les patients. Pour le traitement, des bolus de corticoïdes intraveineux ont été administrés, relayés par une corticothérapie orale et associés à une kinésithérapie motrice et un traitement antiépileptique pour certains patients. L’évolution de nos malades était favorable globalement et on a déploré le décès d'une patiente. L'ADEM est une affection rare du système nerveux central. Les critères diagnostiques sont fondés sur des éléments cliniques et des données d'imagerie. La prise en charge doit être précoce et multidisciplinaire.

P137 – 1499 Acute disseminated encephalomyelitis in children and adolescents: a single center case series

P137 – 1499 Acute disseminated encephalomyelitis in children and adolescents: a single center case series

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.

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.

1503 Acute Disseminated Encephalomyelitis in Children (Eight Cases Reports)

BackgroundAcute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory disorder of the central nervous system, characterized by a widespread demyelination that predominantly involves the white matter of the brain and spinal...