Prognostic indicators in pediatric clinically isolated syndrome. Issue 5 (May 2017)

• Record Type: Journal Article
• Authors: Iaffaldano, Pietro; Simone, Marta; Lucisano, Giuseppe; Ghezzi, Angelo; Coniglio, Gabriella; Brescia Morra, Vincenzo; Salemi, Giuseppe; Patti, Francesco; Lugaresi, Alessandra; Izquierdo, Guillermo; Bergamaschi, Roberto; Cabrera‐Gomez, Jose Antonio; Pozzilli, Carlo; Millefiorini, Enrico; Alroughani, Raed; Boz, Cavit; Pucci, Eugenio; Zimatore, Giovanni Bosco; Sola, Patrizia; Lus, Giacomo; Maimone, Davide; Avolio, Carlo; Cocco, Eleonora; Sajedi, Seyed Aidin; Costantino, Gianfranco; Duquette, Pierre; Shaygannejad, Vahid; Petersen, Thor; Fernández Bolaños, Ricardo; Paolicelli, Damiano; Tortorella, Carla; Spelman, Tim; Margari, Lucia; Amato, Maria Pia; Comi, Giancarlo; Butzkueven, Helmut; Trojano, Maria;
• Other Names: Spitaleri Daniele investigator.; Rottoli Maria Rosa investigator.; Ardito Bonaventura investigator.; Iuliano Gerardo investigator.; Montanari Enrico investigator.; Granieri Enrico investigator.; Tedeschi Gioacchino investigator.; Bertolotto Antonio investigator.; Granella Franco investigator.; Di Battista Giancarlo investigator.; Gallo Paolo investigator.; Cavalla Paola investigator.; Bellantonio Paolo investigator.; De Robertis Francesca investigator.; Durelli Luca investigator.; Scarpini Elio investigator.; Rezzonico Monica investigator.; Protti Alessandra investigator.; Solaro Claudio investigator.; Corea Francesco investigator.; Bosco Antonio investigator.; Vianello Marika investigator.; Ferrò Maria Teresa investigator.; Balgera Roberto investigator.; Grasso Roberta investigator.; De Luca Giovanna investigator.; Farina Deboah investigator.; Travaglini Daniela investigator.; di Ioia Maria investigator.; Di Tommaso Valeria investigator.; Mancinelli Luca investigator.; Pietrolongo Erika investigator.; Hupperts Raymond investigator.; Rio Maria Edite investigator.; Terzi Murat investigator.; Barnett Michael investigator.; Slee Mark investigator.; Van Pesch Vincent investigator.; Savino Aldo investigator.; Lechner‐Scott Jeannette investigator.; Grammond Pierre investigator.; Singhal Bhim investigator.; Zwanikken Cees investigator.; Fiol Marcela investigator.; Patrucco Liliana investigator.; Paine Mark investigator.; McCombefrom Pamela investigator.; Grand'Maison Francois investigator.;
• Is Part Of: Annals of neurology. Volume 81:Issue 5(2017)
• Page Start: 729
• Page End: 739
• Publication Date: 2017-05
• Subjects: Neurology -- Periodicals; Pediatric neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; 616.8
• DOI: 10.1002/ana.24938
• Languages: English
• ISSNs: 0364-5134

Abstract

Abstract : Objective: To assess prognostic factors for a second clinical attack and a first disability‐worsening event in pediatric clinically isolated syndrome (pCIS) suggestive of multiple sclerosis (MS) patients. Methods: A cohort of 770 pCIS patients was followed up for at least 10 years. Cox proportional hazard models and Recursive Partitioning and Amalgamation (RECPAM) tree‐regression were used to analyze data. Results: In pCIS, female sex and a multifocal onset were risk factors for a second clinical attack (hazard ratio [HR], 95% confidence interval [CI] = 1.28, 1.06–1.55; 1.42, 1.10–1.84, respectively), whereas disease‐modifying drug (DMD) exposure reduced this risk (HR, 95% CI = 0.75, 0.60–0.95). After pediatric onset MS (POMS) diagnosis, age at onset younger than 15 years and DMD exposure decreased the risk of a first Expanded Disability Status Scale (EDSS)‐worsening event (HR, 95% CI = 0.59, 0.42–0.83; 0.75, 0.71–0.80, respectively), whereas the occurrence of relapse increased this risk (HR, 95% CI = 5.08, 3.46–7.46). An exploratory RECPAM analysis highlighted a significantly higher incidence of a first EDSS‐worsening event in patients with multifocal or isolated spinal cord or optic neuritis involvement at onset in comparison to those with an isolated supratentorial or brainstem syndrome. A Cox regression model including RECPAM classes confirmed DMD exposure as the most protective factor against EDSS‐worsening events and relapses as the most important risk factor for attaining EDSS worsening. Interpretation: This work represents a step forward in identifying predictors of unfavorable course in pCIS and POMS and supports a protective effect of early DMD treatment in preventing MS development and disability accumulation in this population. Ann Neurol 2017;81:729–739