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The natural course of clinically isolated syndrome in pediatric patients

Cha Gon Lee1, Bolyun Lee2, Jeehun Lee3

  • 1Department of Pediatrics, Eulji General Hospital, College of Medicine, Eulji University, Seoul, Republic of Korea.

Brain & Development
|August 3, 2014
PubMed

Insights

Clinically isolated syndrome (CIS) in children can progress to multiple sclerosis (MS). A second relapse or specific initial symptoms like brain stem dysfunction strongly predict MS development in pediatric patients.

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Clinical Demyelinating Diseases

Background:

  • Clinically isolated syndrome (CIS) is the first episode of central nervous system (CNS) symptoms suggesting demyelination in children.
  • Diagnostic criteria for pediatric CNS demyelination were established in 2007.
  • Understanding the natural course and prognostic factors of pediatric CIS is crucial.

Purpose of the Study:

  • To investigate the natural course of pediatric CIS.
  • To identify factors influencing the prognosis of pediatric CIS.
  • To determine predictors of conversion to multiple sclerosis (MS) in children.

Main Methods:

  • Retrospective review of medical records of pediatric patients (<18 years) with CNS events from 1997-2008.
  • Analysis of clinical presentations, demographic data, and follow-up information.
  • Assessment of conversion to MS based on established diagnostic criteria.

Main Results:

  • 32 pediatric CIS patients identified (mean age 10.0±4.1 years).
  • Optic neuritis was the most common initial presentation.
  • 50% experienced a second demyelinating event; 34% developed childhood MS.
  • Asymptomatic MRI lesions and CSF oligoclonal bands did not predict MS conversion.

Conclusions:

  • A second relapse and initial multifocal or specific CNS dysfunction (brain stem, cerebellar, cerebral) strongly predict MS development in pediatric CIS (p=0.002).
  • Predicting progression of pediatric CIS to chronic demyelinating disease remains challenging despite improved understanding.
Abstract

Keywords:
Autoimmune diseaseChildDemyelinating diseaseMultiple sclerosisNeuromyelitis optica

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