• X. Montalban
Part of the Topics in Neuroscience book series (TOPNEURO)


Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system (CNS). Three major clinical courses have been identified in MS: relapsing-remitting MS (RRMS), characterized by exacerbations with subsequent total or partial remission of symptoms; secondary progressive MS (SPMS), in which progression follows an initial RRMS phase; and primary progressive MS (PPMS), a progressive form without relapses or remissions [1]. Although by definition PPMS is clinically different from the relapsing forms, in the past, the SPMS and PPMS forms were grouped together as “chronic progressive” (CPMS) MS. However, in the first magnetic resonance imaging (MRI) study in which PPMS was specifically investigated, it was shown that patients with PPMS, despite marked disability, had fewer and smaller gadolinium-enhancing cerebral MRI lesions than patients with SPMS [2, 3]. Furthermore, a more recent study of a large cohort of PPMS patients from six European centres has shown clear differences in MRI appearances between PPMS and SPMS [4]. Few papers have addressed the pathological differences between PPMS and the relapsing forms; inflammation, though less marked, is also obviously present in PPMS lesions [5]. Although a small number of patients with PPMS could have a distinct pathological pattern, the samples studied are small, and some cases of PPMS are indistinguishable from RR forms of MS [6]. While MS is generally associated with the haplotype A3-B7-DR2 (15)-DQw6, this occurs predominantly with the RR form of the disease.


Multiple Sclerosis Experimental Autoimmune Encephalomyelitis Experimental Allergic Encephalomyelitis Multiple Sclerosis Lesion Progressive Multiple Sclerosis 


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© Springer-Verlag Italia, Milano 2002

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  • X. Montalban

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