Primary Progressive Multiple Sclerosis Evolving from Radiologically Isolated Syndrome

Orhun H. Kantarci, Christine Lebrun, Aksel Siva, Mark B. Keegan, Christina J. Azevedo, Matilde Inglese, Mar Tintoré, Braeden D. Newton, Francoise Durand-Dubief, Maria Pia Amato, Nicola De Stefano, Maria Pia Sormani, Daniel Pelletier, Darin T. Okuda

Research output: Contribution to journalArticle

60 Citations (Scopus)

Abstract

Objective The aim of this work was to evaluate the preprogressive phase in subjects with radiologically isolated syndrome (RIS) who evolve to primary progressive multiple sclerosis (PPMS). Methods A multicenter RIS cohort was previously established. Demographic, clinical, and radiological characteristics of subjects with RIS that evolved directly to PPMS were compared to those that developed a relapsing disease course from onset (clinically isolated syndrome [CIS] or relapsing-remitting MS) and were also compared to two other population- and clinic-based PPMS cohorts. Results Of the 453 subjects with RIS, 128 evolved to symptomatic MS during the follow-up (113 developed a first acute clinical event consistent with CIS/MS, 15 evolved to PPMS). PPMS prevalence (11.7%) and onset age (mean ± standard deviation; 49.1 ± 12.1) in the RIS group were comparable to other PPMS populations (p > 0.05). Median time to PPMS was 3.5 years (range, 1.6-5.4). RIS evolved to PPMS more commonly in men (p = 0.005) and at an older age (p <0.001) when compared to CIS/MS, independent of follow-up duration. Subjects who evolved to PPMS had more spinal cord lesions (100%) before symptomatic evolution than those that developed CIS/MS (64%) and those that remained asymptomatic (23%) within the follow-up period (P = 0.005). Other MRI characteristics in the preprogressive phase of PPMS were indistinguishable from CIS/MS. Interpretation Subjects with RIS evolve to PPMS at the same frequency as expected from general MS populations in an age-dependent manner. Besides age, unequivocal presence of spinal cord lesions and being male predicted evolution to PPMS. Our findings further suggest that RIS is biologically part of the MS spectrum.

Original languageEnglish (US)
Pages (from-to)288-294
Number of pages7
JournalAnnals of Neurology
Volume79
Issue number2
DOIs
StatePublished - Feb 1 2016

Fingerprint

Chronic Progressive Multiple Sclerosis
Spinal Cord
Population
Age of Onset

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Kantarci, O. H., Lebrun, C., Siva, A., Keegan, M. B., Azevedo, C. J., Inglese, M., ... Okuda, D. T. (2016). Primary Progressive Multiple Sclerosis Evolving from Radiologically Isolated Syndrome. Annals of Neurology, 79(2), 288-294. https://doi.org/10.1002/ana.24564

Primary Progressive Multiple Sclerosis Evolving from Radiologically Isolated Syndrome. / Kantarci, Orhun H.; Lebrun, Christine; Siva, Aksel; Keegan, Mark B.; Azevedo, Christina J.; Inglese, Matilde; Tintoré, Mar; Newton, Braeden D.; Durand-Dubief, Francoise; Amato, Maria Pia; De Stefano, Nicola; Sormani, Maria Pia; Pelletier, Daniel; Okuda, Darin T.

In: Annals of Neurology, Vol. 79, No. 2, 01.02.2016, p. 288-294.

Research output: Contribution to journalArticle

Kantarci, OH, Lebrun, C, Siva, A, Keegan, MB, Azevedo, CJ, Inglese, M, Tintoré, M, Newton, BD, Durand-Dubief, F, Amato, MP, De Stefano, N, Sormani, MP, Pelletier, D & Okuda, DT 2016, 'Primary Progressive Multiple Sclerosis Evolving from Radiologically Isolated Syndrome', Annals of Neurology, vol. 79, no. 2, pp. 288-294. https://doi.org/10.1002/ana.24564
Kantarci OH, Lebrun C, Siva A, Keegan MB, Azevedo CJ, Inglese M et al. Primary Progressive Multiple Sclerosis Evolving from Radiologically Isolated Syndrome. Annals of Neurology. 2016 Feb 1;79(2):288-294. https://doi.org/10.1002/ana.24564
Kantarci, Orhun H. ; Lebrun, Christine ; Siva, Aksel ; Keegan, Mark B. ; Azevedo, Christina J. ; Inglese, Matilde ; Tintoré, Mar ; Newton, Braeden D. ; Durand-Dubief, Francoise ; Amato, Maria Pia ; De Stefano, Nicola ; Sormani, Maria Pia ; Pelletier, Daniel ; Okuda, Darin T. / Primary Progressive Multiple Sclerosis Evolving from Radiologically Isolated Syndrome. In: Annals of Neurology. 2016 ; Vol. 79, No. 2. pp. 288-294.
@article{649020cc25144e488603ff1ef7740f1a,
title = "Primary Progressive Multiple Sclerosis Evolving from Radiologically Isolated Syndrome",
abstract = "Objective The aim of this work was to evaluate the preprogressive phase in subjects with radiologically isolated syndrome (RIS) who evolve to primary progressive multiple sclerosis (PPMS). Methods A multicenter RIS cohort was previously established. Demographic, clinical, and radiological characteristics of subjects with RIS that evolved directly to PPMS were compared to those that developed a relapsing disease course from onset (clinically isolated syndrome [CIS] or relapsing-remitting MS) and were also compared to two other population- and clinic-based PPMS cohorts. Results Of the 453 subjects with RIS, 128 evolved to symptomatic MS during the follow-up (113 developed a first acute clinical event consistent with CIS/MS, 15 evolved to PPMS). PPMS prevalence (11.7{\%}) and onset age (mean ± standard deviation; 49.1 ± 12.1) in the RIS group were comparable to other PPMS populations (p > 0.05). Median time to PPMS was 3.5 years (range, 1.6-5.4). RIS evolved to PPMS more commonly in men (p = 0.005) and at an older age (p <0.001) when compared to CIS/MS, independent of follow-up duration. Subjects who evolved to PPMS had more spinal cord lesions (100{\%}) before symptomatic evolution than those that developed CIS/MS (64{\%}) and those that remained asymptomatic (23{\%}) within the follow-up period (P = 0.005). Other MRI characteristics in the preprogressive phase of PPMS were indistinguishable from CIS/MS. Interpretation Subjects with RIS evolve to PPMS at the same frequency as expected from general MS populations in an age-dependent manner. Besides age, unequivocal presence of spinal cord lesions and being male predicted evolution to PPMS. Our findings further suggest that RIS is biologically part of the MS spectrum.",
author = "Kantarci, {Orhun H.} and Christine Lebrun and Aksel Siva and Keegan, {Mark B.} and Azevedo, {Christina J.} and Matilde Inglese and Mar Tintor{\'e} and Newton, {Braeden D.} and Francoise Durand-Dubief and Amato, {Maria Pia} and {De Stefano}, Nicola and Sormani, {Maria Pia} and Daniel Pelletier and Okuda, {Darin T.}",
year = "2016",
month = "2",
day = "1",
doi = "10.1002/ana.24564",
language = "English (US)",
volume = "79",
pages = "288--294",
journal = "Annals of Neurology",
issn = "0364-5134",
publisher = "John Wiley and Sons Inc.",
number = "2",

}

TY - JOUR

T1 - Primary Progressive Multiple Sclerosis Evolving from Radiologically Isolated Syndrome

AU - Kantarci, Orhun H.

AU - Lebrun, Christine

AU - Siva, Aksel

AU - Keegan, Mark B.

AU - Azevedo, Christina J.

AU - Inglese, Matilde

AU - Tintoré, Mar

AU - Newton, Braeden D.

AU - Durand-Dubief, Francoise

AU - Amato, Maria Pia

AU - De Stefano, Nicola

AU - Sormani, Maria Pia

AU - Pelletier, Daniel

AU - Okuda, Darin T.

PY - 2016/2/1

Y1 - 2016/2/1

N2 - Objective The aim of this work was to evaluate the preprogressive phase in subjects with radiologically isolated syndrome (RIS) who evolve to primary progressive multiple sclerosis (PPMS). Methods A multicenter RIS cohort was previously established. Demographic, clinical, and radiological characteristics of subjects with RIS that evolved directly to PPMS were compared to those that developed a relapsing disease course from onset (clinically isolated syndrome [CIS] or relapsing-remitting MS) and were also compared to two other population- and clinic-based PPMS cohorts. Results Of the 453 subjects with RIS, 128 evolved to symptomatic MS during the follow-up (113 developed a first acute clinical event consistent with CIS/MS, 15 evolved to PPMS). PPMS prevalence (11.7%) and onset age (mean ± standard deviation; 49.1 ± 12.1) in the RIS group were comparable to other PPMS populations (p > 0.05). Median time to PPMS was 3.5 years (range, 1.6-5.4). RIS evolved to PPMS more commonly in men (p = 0.005) and at an older age (p <0.001) when compared to CIS/MS, independent of follow-up duration. Subjects who evolved to PPMS had more spinal cord lesions (100%) before symptomatic evolution than those that developed CIS/MS (64%) and those that remained asymptomatic (23%) within the follow-up period (P = 0.005). Other MRI characteristics in the preprogressive phase of PPMS were indistinguishable from CIS/MS. Interpretation Subjects with RIS evolve to PPMS at the same frequency as expected from general MS populations in an age-dependent manner. Besides age, unequivocal presence of spinal cord lesions and being male predicted evolution to PPMS. Our findings further suggest that RIS is biologically part of the MS spectrum.

AB - Objective The aim of this work was to evaluate the preprogressive phase in subjects with radiologically isolated syndrome (RIS) who evolve to primary progressive multiple sclerosis (PPMS). Methods A multicenter RIS cohort was previously established. Demographic, clinical, and radiological characteristics of subjects with RIS that evolved directly to PPMS were compared to those that developed a relapsing disease course from onset (clinically isolated syndrome [CIS] or relapsing-remitting MS) and were also compared to two other population- and clinic-based PPMS cohorts. Results Of the 453 subjects with RIS, 128 evolved to symptomatic MS during the follow-up (113 developed a first acute clinical event consistent with CIS/MS, 15 evolved to PPMS). PPMS prevalence (11.7%) and onset age (mean ± standard deviation; 49.1 ± 12.1) in the RIS group were comparable to other PPMS populations (p > 0.05). Median time to PPMS was 3.5 years (range, 1.6-5.4). RIS evolved to PPMS more commonly in men (p = 0.005) and at an older age (p <0.001) when compared to CIS/MS, independent of follow-up duration. Subjects who evolved to PPMS had more spinal cord lesions (100%) before symptomatic evolution than those that developed CIS/MS (64%) and those that remained asymptomatic (23%) within the follow-up period (P = 0.005). Other MRI characteristics in the preprogressive phase of PPMS were indistinguishable from CIS/MS. Interpretation Subjects with RIS evolve to PPMS at the same frequency as expected from general MS populations in an age-dependent manner. Besides age, unequivocal presence of spinal cord lesions and being male predicted evolution to PPMS. Our findings further suggest that RIS is biologically part of the MS spectrum.

UR - http://www.scopus.com/inward/record.url?scp=84958106126&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84958106126&partnerID=8YFLogxK

U2 - 10.1002/ana.24564

DO - 10.1002/ana.24564

M3 - Article

C2 - 26599831

AN - SCOPUS:84958106126

VL - 79

SP - 288

EP - 294

JO - Annals of Neurology

JF - Annals of Neurology

SN - 0364-5134

IS - 2

ER -