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Published on: June 30, 2014
Pediatric Multiple Sclerosis Severity Score in a large US cohort.
Jonathan D Santoro1, Michael Waltz2, Greg Aaen2
1From Partners Pediatric Multiple Sclerosis Center (J.D.S., T.C.), Massachusetts General Hospital; Harvard Medical School (J.D.S.), Boston, MA; Pediatric Multiple Sclerosis and Related Disorders Program at Boston Children's Hospital (J.D.S., L.B., M.G.), MA; Children's Hospital Los Angeles (J.D.S.); Keck School of Medicine at the University of Southern California (J.D.S.), Los Angeles; Data Coordinating and Analysis Center (M.W., S.R., J.R., T.C.C.), University of Utah, Salt Lake City; Pediatric Multiple Sclerosis Center (G.A.), Loma Linda University Children's Hospital, CA; Pediatric MS Center at NYU Langone Health (A.B., L.K.), New York, NY; Washington University (M.S.G., S.M.), St. Louis, MO; Pediatric Multiple Sclerosis Center (J.S.G.), University of California San Diego; UAB Center for Pediatric-Onset Demyelinating Disease (Y.H., J.N.), University of Alabama at Birmingham; The Blue Bird Circle Clinic for Multiple Sclerosis (T.L.), Texas Children's Hospital, Baylor College of Medicine, Houston; Mellen Center for Multiple Sclerosis (M.M., M. Rensel), Cleveland Clinic, OH; Mayo Clinic Pediatric Multiple Sclerosis Center (M. Rodriguez, J.-M.T.), Mayo Clinic, Rochester, MN; Rocky Mountain Multiple Sclerosis Center (T.S.), Children's Hospital Colorado, University of Colorado at Denver, Aurora; Pediatric Multiple Sclerosis Center (E.W.), University of California San Francisco; Jacobs Pediatric Multiple Sclerosis Center (B.W.-G.), State University of New York at Buffalo; and Department of Neurology (B.F.H.), Stanford University School of Medicine, Palo Alto, CA. jdsantoro@chla.usc.edu tchitnis@partners.org.
Pediatric-onset multiple sclerosis (POMS) shows slower disability progression than adult-onset MS. A Pediatric Multiple Sclerosis Severity Score (Ped-MSSS) may offer an alternative to the Expanded Disability Status Scale (EDSS) for assessing disease severity.
Area of Science:
- Neurology
- Pediatric Neurology
- Clinical Research
Background:
- Pediatric-onset multiple sclerosis (POMS) represents a distinct disease course compared to adult-onset MS.
- Understanding disease severity and disability progression in POMS is crucial for effective management.
- Existing disability scales may require optimization for pediatric populations.
Purpose of the Study:
- To characterize disease severity and disability distribution in POMS.
- To develop and validate an optimized modeling scale for measuring disability in POMS.
- To compare disability progression in POMS with adult-onset MS.
Main Methods:
- Multicenter retrospective analysis of disability scores in 873 individuals with POMS.
- Stratification of patients by time from symptom onset to Expanded Disability Status Scale (EDSS) assessment.
- Calculation of Pediatric Multiple Sclerosis Severity Score (Ped-MSSS) based on established criteria.
Main Results:
- POMS patients exhibited lower EDSS scores compared to adult-onset MS cohorts.
- Slower progression of Ped-MSSS was observed with increasing time after diagnosis.
- Motor relapses and Year 1 EDSS were significant predictors of disease progression.
- Symbol Digit Modalities Test (SDMT) scores correlated inversely with disease duration.
Conclusions:
- Individuals with POMS generally experience less severe disability progression than those with adult-onset MS.
- The Ped-MSSS model shows promise as an alternative or complementary tool to EDSS for assessing POMS severity.
- Further validation of the Ped-MSSS is recommended for clinical application.
