The Kurtzke EDSS rank stability increases 4 years after the onset of multiple sclerosis: results from the MSBase Registry

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Hughes, Stella
Spelman, Timothy
Trojano, Maria
Lugaresi, Alessandra
Izquierdo, Guillermo
Grand'Maison, Francois
Duquette, Pierre
Girard, Marc
Grammond, Pierre
Oreja-Guevara, Celia
Hupperts, Raymond
Boz, Cavit
Bergamaschi, Roberto
Giuliani, Giorgio
Rio, Maria Edite
Lechner-Scott, Jeannette
van Pesch, Vincent
Iuliano, Gerardo
Fiol, Marcela
Verheul, Freek
Barnett, Michael
Slee, Mark
Herbert, Joseph
Kister, Ilya
Vella, Norbert
Moore, Fraser
Petkovska-Boskova, Tatjana
Shaygannejad, Vahid
Jokubaitis, Vilija
McDonnell, Gavin
Hawkins, Stanley
Kee, Frank
Gray, Orla
Butzkueven, Helmut
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Background The Expanded Disability Status Scale (EDSS) is widely used to rate multiple sclerosis (MS) disability, but lack of disease duration information limits utility in assessing severity. EDSS ranking at specific disease durations was used to devise the MS Severity Score, which is gaining popularity for predicting outcomes. As this requires validation in longitudinal cohorts, we aimed to assess the utility of EDSS ranking as a predictor of 5-year outcome in the MSBase Registry. Methods Rank stability of EDSS over time was examined in the MSBase Registry, a large multicentre MS cohort. Scores were ranked for 5-year intervals, and correlation of rank across intervals was assessed using Spearman's rank correlation. EDSS progression outcomes at 10 years were disaggregated by 5-year EDSS scores. Results Correlation coefficients for EDSS rank over 5-year intervals increased with MS duration: years 1-6=0.55, years 4-9=0.74, years 7-12=0.80 and years 10-15=0.83. EDSS progression risk at 10 years after onset was highly dependent on EDSS at 5 years; one-point progression risk was greater for EDSS score of >2 than =2. Two-point progression was uncommon for EDSS score of <2 and more common at EDSS score of 4. Conclusions EDSS rank stability increases with disease duration, probably due to reduced relapses and less random variation in later disease. After 4 years duration, EDSS rank was highly predictive of EDSS rank 5 years later. Risk of progression by 10 years was highly dependent on EDSS score at 5 years duration. We confirm the utility of EDSS ranking to predict 5-year outcome in individuals 4 years after disease onset.

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Journal of Neurology, Neurosurgery and Psychiatry

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Biomedical and clinical sciences


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