The pathology of primary progressive multiple sclerosis

2004 ◽  
Vol 10 (3_suppl) ◽  
pp. S23-S30 ◽  
Author(s):  
Claudia Lucchinetti ◽  
Wolfgang Bruck
Keyword(s):  
Multiple Sclerosis ◽  
Axonal Injury ◽  
Treatment Strategies ◽  
Progressive Multiple Sclerosis ◽  
Primary Progressive Multiple Sclerosis ◽  
Limited Information ◽  
Primary Progressive ◽  
Secondary Progressive ◽  
Relapsing Remitting ◽  
Focal Demyelination

The patho logical hallmark of chronic multiple sclerosis includes focal demyelination, gliosis, inflammation and axonal injury. There is limited information on whether these pathological features differ across the clinical pheno types of the disease (relapsing-remitting, secondary progressive, and primary progressive). This review will focus on the patho logical aspects of PPMS and pathogenic implications. A better understanding of the differences in PPMS pathology and patho genesis will lead to more effective treatment strategies.

The pathology of primary progressive multiple sclerosis

2002 ◽  
Vol 8 (2) ◽  
pp. 93-97 ◽  
Author(s):  
W Brück ◽  
C Lucchinetti ◽  
H Lassmann
Keyword(s):  
Multiple Sclerosis ◽  
Demyelinating Disease ◽  
Current Knowledge ◽  
Progressive Multiple Sclerosis ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Secondary Progressive ◽  
Relapsing Remitting ◽  
Underlying Pathology ◽  
Clinical Subtype

The present review will focus on the current knowledge of the pathology of primary progressive multiple sclerosis lesions. Multiple sclerosis (MS) is an inflammatory demyelinating disease with a broad clinical variability. The main disease courses are relapsing-remitting, secondary progressive and primary progressive MS. Pathological studies examining the specific underlying pathology of a defined clinical subtype are rare. Here, we focus on the phatological characteristics of the MS lesions and summarize the current findings of the phatology of primary progressive MS with respect to inflammation, oligodendrocyte/myelin pathology, axon destruction and immunopathology of the lesions.

Primary progressive multiple sclerosis: cerebrospinal fluid considerations

2004 ◽  
Vol 10 (3_suppl) ◽  
pp. S31-S35
Author(s):  
Mark S Freedman
Keyword(s):  
Multiple Sclerosis ◽  
Cerebrospinal Fluid ◽  
State Of The Art ◽  
Disease Process ◽  
Progressive Multiple Sclerosis ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Secondary Progressive ◽  
Current State ◽  
Relapsing Remitting

Diagnosing the ‘primary progressive’ form of multiple sclerosis (PPMS) requires assurance that other conditions that might cause a chronic inflammatory neurodegenerative central nervous system (CNS) disease have been ruled out. Both imaging and pathological studies have shown that this form of MS tends to be less inflammatory compared with either the relapsing-remitting or secondary progressive types. There are therefore many conditions that cause a slowly progressive wasting of the C NS that might be confused with MS. The new MS diagnostic scheme has made the presence of ‘typical’ MS abnormalities in the cerebrospinal fluid (C SF) a mandatory first criterion, but there may well be individuals that still have PPMS even in the absence of a typical MS C SF. Here we explore what the C SF can tell about an individual’s disease process and outline the current state of the art in terms of C SF analysis. Used properly, the C SF can be very helpful in clarifying a diagnosis of PPMS.

Prodrome in relapsing-remitting and primary progressive multiple sclerosis

2019 ◽  
Vol 26 (7) ◽  
pp. 1032-1036 ◽  
Author(s):  
J. M. A. Wijnands ◽  
F. Zhu ◽  
E. Kingwell ◽  
Y. Zhao ◽  
C. Evans ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Progressive Multiple Sclerosis ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Relapsing Remitting

scholarly journals Retrospective unbiased plasma lipidomic of progressive multiple sclerosis patients-identifies lipids discriminating those with faster clinical deterioration

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Mario Amatruda ◽  
Maria Petracca ◽  
Maureen Wentling ◽  
Benjamin Inbar ◽  
Kamilah Castro ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Disease Progression ◽  
Progressive Multiple Sclerosis ◽  
Primary Progressive Multiple Sclerosis ◽  
Disease Course ◽  
Primary Progressive ◽  
T2 Lesions ◽  
Relapsing Remitting ◽  
One Year ◽  
Multiple Sclerosis Patients

Abstract The disease course of patients with a confirmed diagnosis of primary progressive multiple sclerosis (PPMS) is uncertain. In an attempt to identify potential signaling pathways involved in the evolution of the disease, we conducted an exploratory unbiased lipidomic analysis of plasma from non-diseased controls (n = 8) and patients with primary progressive MS (PPMS, n = 19) and either a rapid (PPMS-P, n = 9) or slow (PPMS-NP, n = 10) disease course based on worsening disability and/or MRI-visible appearance of new T2 lesions over a one-year-assessment. Partial least squares-discriminant analysis of the MS/MSALL lipidomic dataset, identified lipids driving the clustering of the groups. Among these lipids, sphingomyelin-d18:1/14:0 and mono-hexosylceramide-d18:1/20:0 were differentially abundant in the plasma of PPMS patients compared to controls and their levels correlated with MRI signs of disease progression. Lyso-phosphatidic acid-18:2 (LPA-18:2) was the only lipid with significantly lower abundance in PPMS patients with a rapidly deteriorating disease course, and its levels inversely correlated with the severity of the neurological deficit. Decreased levels of LPA-18:2 were detected in patients with more rapid disease progression, regardless of therapy and these findings were validated in an independent cohort of secondary progressive (SPMS) patients, but not in a third cohorts of relapsing–remitting (RRMS) patients. Collectively, our analysis suggests that sphingomyelin-d18:1/14:0, mono-hexosylceramide-d18:1/20:0, and LPA-18:2 may represent important targets for future studies aimed at understanding disease progression in MS.

Mitoxantrone as a potential therapy for primary progressive multiple sclerosis

2004 ◽  
Vol 10 (3_suppl) ◽  
pp. S58-S61 ◽  
Author(s):  
Olaf Stüve ◽  
Mariko Kita ◽  
Daniel Pelletier ◽  
Robert J Fox ◽  
Jerome Stone ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Western Europe ◽  
Progressive Multiple Sclerosis ◽  
Primary Progressive Multiple Sclerosis ◽  
Pharmacological Properties ◽  
Phase 2 ◽  
Double Blind ◽  
Primary Progressive ◽  
Secondary Progressive ◽  
Phase 2 Trial

Mitoxantrone (Novantrone®) was the first drug approved in western Europe and North A merica for treatment of secondary progressive multiple sclerosis (SPMS) and progressive relapsing MS (PRMS). Pharmacological properties of mitoxantrone, its role in SPMS, the study rational and design of an ongoing multi-centre, double blind, randomized, placebo -controlled phase 2 trial will be outlined in this article.

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