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The Multiple Sclerosis Performance Test MSPT: An iPad-Based Disability Assessment Tool
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.
Insights
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.
Objective:
To characterize disease severity and distribution of disability in pediatric-onset multiple sclerosis (POMS) and to develop an optimized modeling scale for measuring disability, we performed a multicenter retrospective analysis of disability scores in 873 persons with POMS over time and compared this to previously published data in adults with multiple sclerosis (MS).
Methods:
This was a retrospective analysis of prospectively collected data collected from 12 centers of the US Network of Pediatric MS Centers. Patients were stratified by the number of years from first symptoms of MS to Expanded Disability Status Scale (EDSS) assessment and an MS severity score (Pediatric Multiple Sclerosis Severity Score [Ped-MSSS]) was calculated per criteria developed by Roxburgh et al. in 2005.
Results:
In total, 873 patients were evaluated. In our cohort, 52%, 19.4%, and 1.5% of all patients at any time point reached an EDSS of 2.0, 3.0, and 6.0. Comparison of our Ped-MSSS scores and previously published adult Multiple Sclerosis Severity Scores (MSSS) showed slower progression of Ped-MSSS with increasing gaps between higher EDSS score and years after diagnosis. Decile scores in our POMS cohort for EDSS of 2.0, 3.0, and 6.0 were 8.00/9.46/9.94, 7.86/9.39/9.91, and 7.32/9.01/9.86 at 2, 5, and 10 years, respectively. Notable predictors of disease progression in both EDSS and Ped-MSSS models were ever having a motor relapse and EDSS at year 1. Symbol Digit Modalities Test (SDMT) scores were inversely correlated with duration of disease activity and cerebral functional score.
Conclusions:
Persons with POMS exhibit lower EDSS scores compared to persons with adult-onset MS. Use of a Ped-MSSS model may provide an alternative to EDSS scoring in clinical assessment of disease severity and disability accrual.

