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

Abstract

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.

Immediate and Short-term Outcome of Acute Disseminated Encephalomyelitis (ADEM) after corticosteroid therapy

“…Disturbed consciousness, motor impairment and seizure are frequently found in many series including ours. 12 Early studies of ADEM reported mortality rates up to 20% with a high incidence of neurological sequele in survivors. [13][14][15][16] In our study mortality was nil.…”

Update on Acute Disseminated Encephalomyelitis in Children and Adolescents

“…Even though most children show excellent clinical recovery and normalization of MRI findings, neurological sequelae have been observed [ 59 ], specifically in children before 5 years of age [ 60 ]. The most common sequelae include cognitive impairment, epilepsy, visual and motor deficits (weakness, spasticity, ataxia), and impairment in speech.…”

Use of Cyclophosphamide in a Child With Fulminant Acute Disseminated Encephalomyelitis

“…Intravenous immunoglobulin could be used as a second-line treatment, particularly in fulminant acute disseminated encephalomyelitis cases who did not respond to corticosteroids. 8 Abdrabou et al 9 reported that patients who received steroids on the first day and intravenous immunoglobulin during the first week of symptom onset had a better outcome. If there is a concern of infection, then intravenous immunoglobulin could be used as an alternative to corticosteroids.…”