Acute Disseminated Encephalomyelitis in PICU (original) (raw)
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Iranian Journal of Child Neurology, 2019
Objectives 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. Materials & Methods 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. Results Overall, 29 children diagnosed as ADEM were studied in terms of demographic characteristics, clinical manifestations and laboratory findings in two groups according to their r...
Electronic physician, 2016
Introduction: Acute 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. Methods: This 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. Results: Eighteen 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). Conclusion: The 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.
Srpski arhiv za celokupno lekarstvo
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...
Acute disseminated encephalomyelitis in children: one medical center experience
Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi
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...
Acute disseminated encephalomyelitis: an evaluation of 15 cases in childhood
PubMed, 2013
To describe our experience with acute disseminated encephalomyelitis (ADEM) and the relationships between the clinical course, magnetic resonance imaging (MRI) findings and treatment, a retrospective record review was conducted of 15 children who were admitted with the diagnosis of ADEM during the period 2004-2010. Their ages ranged between 2 and 13 years. Patients presented most often with ataxia (53.3%) and secondly with weakness and headache (46.6%). Myelitis was determined in two patients. Five patients were treated with high-dose intravenous (IV) methylprednisolone followed by oral prednisolone, seven patients were treated with oral prednisolone, one patient was treated with plasmapheresis with IV immunoglobulin (Ig), and one patient was treated with IVIg. We observed recurrence in one patient. ADEM is a demyelinating disorder that is being diagnosed increasingly more as MRI studies are performed more frequently in patients with acute encephalopathy. Early diagnosis and therapy might improve the outcome.
Rare and Atypical Presentations of Acute Disseminated Encephalomyelitis in Children: A Case Series
Journal of Tropical Pediatrics, 2021
Acute disseminated encephalomyelitis (ADEM) is a monophasic demyelinating disorder of central nervous system occurring in children with a wide range of clinical manifestations after infection or vaccination. There are few case reports in literature, describing atypical presentations of ADEM with fever of unknown origin, autonomic dysfunction, complex movement disorders such as myoclonus, dystonia and chorea, acute psychosis and myocarditis. Here, we report four cases of ADEM with atypical features like uniocular blindness, myelin oligodendrocyte glycoprotein antibodies negative multiphasic disseminated encephalomyelitis, ADEM mimicking Guillain-Barre syndrome at presentation and isolated spinal ADEM. Treatment with high-dose steroids elicited an excellent neurological outcome in all patients. A high index of clinical suspicion along-with awareness of atypical features, magnetic resonance imaging and cerebrospinal fluid studies are of paramount importance in establishing ADEM diagnos...
Outcome of children with acute disseminated encephalomyelitis in a tertiary care center in India
The Turkish journal of pediatrics
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated disease of the brain that follows viral infection or vaccination, or even appears spontaneously. The objective of the study was to evaluate the clinical, neuroimaging and laboratory profiles of children with ADEM. Seventeen children admitted to the Pediatric Department of Dayanand Medical College and Hospital, Ludhiana, whose cases were consistent with the diagnosis of ADEM, were included. Their clinical and neuroimaging profiles and outcomes were studied. The most common presenting features were fever (71%), altered sensorium (59%) and headache and convulsions (41%). Brain MRI identified lesions in subcortical white matter (75%) and periventricular white matter (38%). Most patients were treated with corticosteroids. A favorable outcome was seen in 88% of patients. Although 59% of patients had neurologic sequelae at discharge, only one patient had a major neurological deficit at follow-up. Prognosis for survival and o...
Acute Disseminated Encephalomyelitis in Children
PEDIATRICS, 2002
Objective. To describe the epidemiologic, clinical, neuroimaging, and laboratory features; treatment; and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM).
Pattern and Outcome of Acute Disseminated Encephalomyelitis (ADEM)
The Egyptian Journal of Hospital Medicine, 2019
Background: Acute disseminated encephalomyelitis (ADEM) is an immune disease marked by widespread of inflammation in the brain and spinal cord. ADEM typically damages myelin, causing destruction of white matter. It often occurs following a viral infection or vaccination. Its symptoms are similar to multiple sclerosis (MS) and are considered part of the multiple sclerosis borderline diseases. Patients and Methods: This is an observational prospective study done in Bab Al-Shariah University Hospital in period between 31th of December 2017 to 30th of December 2018 on children from birth to 18 years old who were having definite ADEM by neuroradiological study of the brain (MRI brain) presented to the Neuropediatric outpatients clinic or inpatients of Pediatric Department during the period of the study. Those with history suggestive of definite perinatal asphyxia or with other congenital malformation of neurological system were not involved in our study. Results: We found that the most c...
Acute Diseminated Encephalomyelitis: A Case Report and Review of Literature
Acute disseminated encephalomyelitis (ADEM) is an autoimmune and monophasic central nervous system disease that principally affects brain and spinal cord by causing non-vasculitic inflammatory diffuse demyelination. ADEM is one of the rare causes of impaired consciousness in childhood. Incidence of the disease under the age of 20 is 1.5-3 / 100000 per year and it mostly occurs in children between the ages of 5-8 years. Underlying causes aren't defined clearly yet, however, infections and vaccinations are known as predisposing factors for the disease. Multifocal white matter lesions on magnetic resonance imaging (MRI) are characteristic signs of ADEM. In our case, hyperintense lesions in cerebrum were detected on cranial MRI subsequent to impaired consciousness and convulsion. No specific feature was found in cerebrospinal fluid analysis of the patient. In this paper, we present a three-year-old boy who developed ADEM subsequent to viral upper respiratory tract infection and had full recovery after high dose steroid therapy. Keywords: Acute Disseminated Encephalomyelitis; Cranial MRI; Multiple Lesions; High Dose Steroid Treatment.