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Ann Neurol. 2016 Feb;79(2):288-94. doi: 10.1002/ana.24564. Epub 2015 Dec 29.

Primary Progressive Multiple Sclerosis Evolving From Radiologically Isolated Syndrome.

Author information

1
Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN.
2
Hôpital Pasteur, Service de Neurologie, Nice, France.
3
Department of Neurology, Cerrahpasa School of Medicine, University of Istanbul, Istanbul, Turkey.
4
Department of Neurology, Multiple Sclerosis Center, Keck School of Medicine of the University of Southern California, Los Angeles, CA.
5
Mt. Sinai School of Medicine, New York, NY.
6
MS Center of Catalunya Cemcat, Vall d'Hebron Hospital, Barcelona, Spain.
7
Department of Neurology & Neurotherapeutics, Clinical Center for Multiple Sclerosis, Multiple Sclerosis and NeuroImmunology Imaging ProgramUniversity of Texas Southwestern Medical Center, Dallas, TX.
8
Service de Neurologie, Hôpital Neurologique, Bron, France.
9
Department of Neurology, University of Florence, Florence, Italy.
10
Department of Neurology, Neurosurgery and Behavioral Sciences, University of Siena, Siena, Italy.
11
Department of Health Sciences (DISSAL), University of Genoa, Genoa, Italy.

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.

PMID:
26599831
DOI:
10.1002/ana.24564
[Indexed for MEDLINE]

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