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.

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.

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 Symptomatic Therapy and Rehabilitation in Primary Progressive Multiple Sclerosis

2011 ◽  
Vol 2011 ◽  
pp. 1-22 ◽  
Author(s):  
Fary Khan ◽  
Bhasker Amatya ◽  
Lynne Turner-Stokes
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Demyelinating Disease ◽  
Disease Onset ◽  
Functional Independence ◽  
Symptomatic Therapy ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Relapsing Remitting

Multiple sclerosis (MS) is an autoimmune inflammatory demyelinating disease of the central nervous system and a major cause of chronic neurological disability in young adults. Primary progressive MS (PPMS) constitutes about 10% of cases, and is characterized by a steady decline in function with no acute attacks. The rate of deterioration from disease onset is more rapid than relapsing remitting and secondary progressive MS types. Multiple system involvement at onset and rapid early progression have a worse prognosis. PPMS can cause significant disability and impact on quality of life. Recent studies are biased in favour of relapsing remitting patients as treatment is now available for them and they are more likely to be seen at MS clinics. Since prognosis for PPMS is worse than other types of MS, the focus of rehabilitation is on managing disability and enhancing participation, and application of a “neuropalliative” approach as the disease progresses. This chapter presents the symptomatic treatment and rehabilitation for persons with MS, including PPMS. A multidisciplinary approach optimizes the intermediate and long-term medical, psychological and social outcomes in this population. Restoration and maintenance of functional independence and societal reintegration, and issues relating to quality of life are addressed in rehabilitation processes.

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.

scholarly journals B-27 Primary Progressive Multiple Sclerosis in an Older Man

2019 ◽  
Vol 34 (6) ◽  
pp. 973-973
Author(s):  
C Schieszler-Ockrassa ◽  
F Bylsma
Keyword(s):  
Multiple Sclerosis ◽  
Cognitive Dysfunction ◽  
Processing Speed ◽  
Demyelinating Disease ◽  
Progressive Multiple Sclerosis ◽  
Fine Motor ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Motor Dexterity ◽  
One Year

Abstract Objective Multiple sclerosis (MS) is an autoimmune demyelinating disease more common in women than men, usually diagnosed between 20-30 years of age. Approximately 50% of individuals with MS develop cognitive dysfunction, with men and progressive MS subtype cases at higher risk (Beatty & Aupperle, 2002). Mr. Doe’s case is unique because he was diagnosed with primary progressive MS at age 56 and demonstrated only mild cognitive dysfunction. Method Mr. Doe presented to his neurologist with complaints of extreme fatigue, slowed processing, and sensory and motor disturbances. He was seen for neuropsychological evaluation one year after diagnosis and was reassessed one year later. He reported worsening mood including passive suicidal ideation since diagnosis. He reported difficulties with work duties (attorney) and household demands due to gradual motor and sensory disturbances, slowed processing speed, fatigue, and mood disturbance. Results Mr. Doe’s initial neuropsychological assessment revealed variability in auditory working memory, weakness in sustained visual attention, and mild deficits in upper extremity fine motor dexterity. Memory, executive functioning, language, and processing speed were all intact unless a motor component was involved (mild decline after one year). His cognitive performances remained generally stable after one year, but depression, anxiety, and hopelessness levels were all significantly worse. Conclusions Although Mr. Doe’s impairments are extremely mild and somewhat unexpected given the primary progressive MS diagnosis, his gender, and age, the affected domains are consistent with the diagnosis. This case demonstrates the importance of understanding base rates for conditions we assess, but also not ruling out lower base rate conditions.

Sign in / Sign up

Export Citation Format

Share Document