Related Experiment Video
Updated: Jun 16, 2026
Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Evaluation of inflammatory acquired demyelinating syndromes in children: a single-center experience
Huseyin Kilic1, Deniz Mavi2, Beyza Citci Yalcinkaya3
1Department of Pediatric Neurology, Cerrahpasa School of Medicine, Istanbul University-Cerrahpasa, Istanbul, Turkey. kilichuseyin@me.com.
Insights
This study highlights key differences in clinical and neuroimaging features among pediatric acquired demyelinating syndromes (ADS), including acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS). Findings aid in distinguishing these conditions in children.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Acquired demyelinating syndromes (ADS) encompass a spectrum of neurological disorders in children.
- Understanding the distinct clinical and neuroimaging characteristics of pediatric ADS subtypes is crucial for accurate diagnosis and management.
- This study focuses on a Turkish cohort to elucidate these features.
Purpose of the Study:
- To evaluate the clinical and neuroimaging features of pediatric acquired demyelinating syndromes (ADS) in a tertiary pediatric neurology clinic.
- To compare the characteristics of different ADS categories, including acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), clinically isolated syndrome (CIS), and Neuromyelitis Optica Spectrum Disorder (NMOSD).
Main Methods:
- Retrospective cohort study of 42 pediatric patients diagnosed with ADS between 2013 and 2018.
- Analysis of clinical presentation, laboratory findings (e.g., pleocytosis, oligoclonal bands), and neuroimaging (e.g., lesion location).
- Comparison of features between ADEM, MS, CIS, and NMOSD groups.
Main Results:
- ADEM and MS were the most common pediatric ADS categories.
- Children with ADEM presented at a younger age with more frequent encephalopathy and basal ganglia/thalamus lesions compared to MS and CIS.
- Seizures and pleocytosis were more common in ADEM, while oligoclonal bands and periventricular lesions were more frequent in MS.
Conclusions:
- Distinct clinical and neuroimaging profiles differentiate pediatric ADS subtypes, aiding in their classification.
- The findings provide insights into the longitudinal disease course of various ADS categories within a single center.
- Rituximab showed promise in preventing relapses in specific severe cases.
Abstract:
To evaluate the clinical and neuroimaging features of pediatric acquired demyelinating syndromes (ADS) in a tertiary pediatric neurology clinic in Turkey. All children diagnosed with any subset of ADS between 2013 and 2018 were included in this retrospective cohort study. Forty-two patients (21 female) with a median follow-up period of 30 months were included. The median age of the patients at disease onset was 11 years (range 1.5-17 years). The most common pediatric ADS categories according to the International pediatric Multiple Sclerosis Study Group consensus classification criteria were acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS), each of which seen in 15 patients, followed by clinically isolated syndrome (CIS) (n = 11) and Neuromyelitis Optica Spectrum Disorder (NMOSD) (n = 1). At the first clinical event, children with ADEM significantly differed from the children affected by MS and CIS in terms of the following parameters: median age at onset (7 vs. 13.5 and 14.5 years; p < 0.001), encephalopathy (93.3 vs 0% and 0%; p < 0.001), and basal ganglia/thalamus lesions (73.3 vs 9.1% and 9.1%; p < 0.001). The frequency of seizure and pleocytosis were higher in ADEM group than MS group (p < 0.05), whereas oligoclonal bands (p < 0.001) and periventricular white matter lesions (p < 0.01) were more frequently observed in MS patients. Rituximab was used with great success in the prevention of relapses in 3 patients: NMOSD (n = 1), MS (n = 1) and ADEM followed by recurrent optic neuritis (n = 1). Our results define the longitudinal disease course of various ADS categories in a single referral center. In addition, this study compares various clinical, laboratory and neuroimaging features between these ADS categories.
