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Primary progressive multiple sclerosis: part of the MS disease spectrum or separate disease entity?

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Abstract

Multiple sclerosis (MS), the most frequent demyelinating disease, is characterized by a variable disease course. The majority of patients starts with relapsing remitting (RR) disease; approximately 50–60% of these patients progress to secondary progressive (SP) disease. Only about 15% of the patients develop a progressive disease course from onset, termed primary progressive multiple sclerosis (PPMS); the underlying pathogenic mechanisms responsible for onset of the disease with either PPMS or relapsing remitting multiple sclerosis (RRMS) are unknown. Patients with PPMS do not show a female predominance and usually have a later onset of disease compared to patients with RRMS. Monozygous twins can be concordant or discordant for disease courses indicating that the disease course is not only genetically determined. Primary progressive multiple sclerosis and secondary progressive multiple sclerosis (SPMS) share many similarities in imaging and pathological findings. Differences observed among the different disease courses are more of a quantitative than qualitative nature suggesting that the different phenotypes are part of a disease spectrum modulated by individual genetic predisposition and environmental influences. In this review, we summarize the knowledge regarding the clinical, epidemiological, imaging, and pathological characteristics of PPMS and compare those characteristics with RRMS and SPMS.

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Acknowledgments

The authors wish to thank Dr. David Arauju and Mr. Mishkin Derakhshan, McConnell Brain Imaging Centre, Montreal Neurological Institute, McGill University who provided some of the imaging figures.

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Authors and Affiliations

  1. Neuroimmunology Unit, Montreal Neurological Institute, McGill University, Montreal, QC, H3A 2B4, Canada

    Jack Antel

  2. McConnell Brain Imaging Centre, Montreal Neurological Institute, McGill University, 3801 University Street, Montreal, QC, H3A 2B4, Canada

    Samson Antel, Zografos Caramanos & Douglas L. Arnold

  3. Institute of Neuropathology, University Hospital Münster, Domagkstr. 19, 48149, Münster, Germany

    Tanja Kuhlmann

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  1. Jack Antel
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Correspondence to Jack Antel or Tanja Kuhlmann.

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Antel, J., Antel, S., Caramanos, Z. et al. Primary progressive multiple sclerosis: part of the MS disease spectrum or separate disease entity?. Acta Neuropathol 123, 627–638 (2012). https://doi.org/10.1007/s00401-012-0953-0

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