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A long-term follow-up study using IPMSSG criteria in children with CNS demyelination
Shubhangi S Peche1, Amer Alshekhlee, James Kelly
1Department of Pediatrics, Division of Child and Adolescent Neurology, Southern Illinois University, Springfield, Illinois.
Insights
Children initially diagnosed with clinically isolated syndrome are more likely to develop multiple sclerosis (MS) than those with acute disseminated encephalomyelitis (ADEM). Female gender, brain stem/hemispheric dysfunction, and specific MRI findings predict MS development.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Central Nervous System Disorders
Background:
- Pediatric inflammatory demyelinating diseases (PIDDs) present diagnostic challenges.
- The International Pediatrics Multiple Sclerosis Study Group (IPMSSG) criteria aid in classifying these conditions.
- Early identification of children at risk for multiple sclerosis (MS) is crucial.
Purpose of the Study:
- To assess the utility of IPMSSG criteria in children with acute central nervous system demyelination.
- To identify predictors for the future development of multiple sclerosis in pediatric patients.
Main Methods:
- Collected baseline data from 123 children experiencing their first episode of acute CNS demyelination.
- Recorded initial diagnoses using IPMSSG criteria and compared them with final diagnoses after follow-up.
- Utilized multivariate logistic regression to identify predictors of MS development.
Main Results:
- 38.2% met criteria for ADEM, 54.4% for clinically isolated syndrome (CIS), 3.2% for neuromyelitis optica, and 4% had unspecified diagnoses.
- Of those with CIS, 38.8% converted to MS; 8.5% of those with ADEM converted to MS.
- Predictors for MS included female gender (OR 12.44), monofocal dysfunction (OR 24.57), and meeting Callen MRI criteria (OR 122.45).
Conclusions:
- IPMSSG criteria highlight that initial CIS in children carries a higher risk of future MS compared to initial ADEM.
- Female sex, brain stem/hemispheric involvement, and specific MRI findings are significant predictors of MS diagnosis in children.
Objective:
To evaluate the practical application of International Pediatrics Multiple Sclerosis study group definitions in children with inflammatory demyelination of the central nervous system and to identify predictors of multiple sclerosis.
Methods:
Baseline data on 123 children with a first episode of acute central nervous system demyelination were collected. The initial diagnosis according to the International Pediatrics Multiple Sclerosis study group was recorded and compared with final diagnosis.
Results:
Forty-seven (38.2%) children met International Pediatrics Multiple Sclerosis study group criteria for acute disseminated encephalomyelitis and 67 (54.4%) had clinically isolated syndrome at the initial presentation. Four (3.2%) had the diagnosis of neuromyelitis optica and five (4%) did not meet any specific diagnosis per the study group criteria. Clinical follow-up was available on 118 of 123 children (95.9%), with a median of 61.5 months (quartile range 23, 110 months). Conversion from clinically isolated syndrome to multiple sclerosis occurred in 26 of 67 children (38.8%); acute disseminated encephalomyelitis to multiple sclerosis occurred in 4 of 47 children (8.5%). Adjusted multivariate logistic regression analysis for an outcome of future development of multiple sclerosis showed the following predictors: female gender (odds ratio 12.44; 95% confidence interval 1.03-149.3); initial diagnosis of monofocal brain stem or hemispheric dysfunction (odds ratio 24.57; 95% confidence interval 3.06-196.78); and Callen magnetic resonance imaging criteria if met (odds ratio 122.45; 95% confidence interval 16.57-904.57).
Conclusion:
International Pediatrics Multiple Sclerosis study group criteria affirm that children with initial clinically isolated syndrome are more likely to develop future multiple sclerosis compared with those with an acute disseminated encephalomyelitis initial diagnosis. In addition, female gender, brain stem or hemispheric involvement, and Callen magnetic resonance imaging criteria predict the diagnosis of multiple sclerosis.
