Aggressive multiple sclerosis (1): Towards a definition of the phenotype. (August 2020)

• Record Type: Journal Article
• Authors: Iacobaeus, Ellen; Arrambide, Georgina; Amato, Maria Pia; Derfuss, Tobias; Vukusic, Sandra; Hemmer, Bernhard; Tintore, Mar; Brundin, Lou
• Other Names: Berger Joseph non-byline-author.; Boyko Alexey non-byline-author.; Brinar Vesna non-byline-author.; Brownlee Wallace non-byline-author.; Ciccarelli Olga non-byline-author.; Coles Alasdair non-byline-author.; Correale Jorge non-byline-author.; Cutter Gary non-byline-author.; Edan Gilles non-byline-author.; Evangelou Nikolaos non-byline-author.; Fernandez Oscar non-byline-author.; Frederiksen Jette non-byline-author.; Gold Ralf non-byline-author.; Hacohen Yael non-byline-author.; Hartung Hans-Peter non-byline-author.; Hellwig Kerstin non-byline-author.; Hillert Jan non-byline-author.; Imitola Jaime non-byline-author.; Kalincik Tomas non-byline-author.; Kappos Ludwig non-byline-author.; Khoury Samia non-byline-author.; Kim Ho Jin non-byline-author.; Havrdová Eva Kubala non-byline-author.; Liblau Roland non-byline-author.; Lycke Jan non-byline-author.; Montalban Xavier non-byline-author.; Muraro Paolo non-byline-author.; Reingold Stephen non-byline-author.; Schmierer Klaus non-byline-author.; Sellebjerg Finn non-byline-author.; Sørensen Per Soelberg non-byline-author.; Solari Alessandra non-byline-author.; Sormani Maria Pia non-byline-author.; Thompson Alan non-byline-author.; Trapp Bruce non-byline-author.; Tremlett Helen non-byline-author.; Trojano Maria non-byline-author.; Tur Carmen non-byline-author.; Uccelli Antonio non-byline-author.; van Pesch Vincent non-byline-author.; Waubant Emmanuelle non-byline-author.;
• Is Part Of: Multiple sclerosis. Volume 26:Number 9(2020)
• Page Start: 1031
• Page End: 1044
• Publication Date: 2020-08
• Subjects: Aggressive -- highly active -- multiple sclerosis -- disability -- relapsing/remitting -- observational studies; Central nervous system -- Diseases -- Periodicals; Myelin sheath -- Diseases -- Periodicals; Inflammation -- Periodicals; Multiple sclerosis -- Periodicals; Central Nervous System Diseases -- Periodicals; Demyelinating Diseases -- Periodicals; Inflammation -- Periodicals; Multiple Sclerosis -- Periodicals; Système nerveux central -- Maladies -- Périodiques; Gaine de myéline -- Maladies -- Périodiques; Inflammation (Pathologie) -- Périodiques; Sclérose en plaques -- Périodiques; Electronic journals; 616.834005
• DOI: 10.1177/1352458520925369
• Languages: English
• ISSNs: 1352-4585

Abstract

While the major phenotypes of multiple sclerosis (MS) and relapsing–remitting, primary and secondary progressive MS have been well characterized, a subgroup of patients with an active, aggressive disease course and rapid disability accumulation remains difficult to define and there is no consensus about their management and treatment. The current lack of an accepted definition and treatment guidelines for aggressive MS triggered a 2018 focused workshop of the European Committee for Treatment and Research in Multiple Sclerosis (ECTRIMS) on aggressive MS. The aim of the workshop was to discuss approaches on how to describe and define the disease phenotype and its treatments. Unfortunately, it was not possible to come to consensus on a definition because of unavailable data correlating severe disease with imaging and molecular biomarkers. However, the workshop highlighted the need for future research needed to define this disease subtype while also focusing on its treatment and management. Here, we review previous attempts to define aggressive MS and present characteristics that might, with additional research, eventually help characterize it. A companion paper summarizes data regarding treatment and management.