Lesion enhancement diminishes with time in primary progressive multiple sclerosis

2010 ◽  
Vol 16 (3) ◽  
pp. 317-324 ◽  
Author(s):  
Z. Khaleeli ◽  
O. Ciccarelli ◽  
K. Mizskiel ◽  
D. Altmann ◽  
DH Miller ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Progressive Multiple Sclerosis ◽  
Primary Progressive Multiple Sclerosis ◽  
Linear Regression Models ◽  
Mixed Effect ◽  
Gadolinium Dtpa ◽  
Primary Progressive ◽  
Inflammatory Phase ◽  
Brain And Spinal Cord

Fewer gadolinium-enhancing lesions are seen in established primary progressive multiple sclerosis (PPMS) compared with other subtypes. Previously, we found unexpectedly high enhancement levels in early PPMS (42%), suggesting an early inflammatory phase. The objective of this study was to investigate whether this level of enhancement was maintained, and whether it influenced clinical progression, over 5 years. Forty-five patients with PPMS, within 5 years of onset, were scored on the Expanded Disability Status Scale (EDSS), Multiple Sclerosis Functional Composite (MSFC) and its subtests (including the timed walk test [TWT]) 6-monthly for 3 years, and at 5 years. T1-weighted brain and spinal cord images after triple dose gadolinium—DTPA, and T2-weighted brain sequences were also acquired. A mixed effect logistic model evaluated change in the percentage of patients with enhancing lesions. Ordinal logistic and multiple linear regression models identified predictors of progression, adjusted for T2 lesion load. The percentage of patients with enhancing lesions in the brain and spinal cord declined over 5 years ( p = 0.03). Among patients with enhancement, more enhancing lesions at baseline predicted greater decline in mobility on the TWT over 5 years ( p = 0.02). In conclusion, a proportion of patients with PPMS may undergo an early inflammatory phase, which has some impact on subsequent mobility.

scholarly journals Ongoing microstructural changes in the cervical cord underpin disability progression in early primary progressive multiple sclerosis

2020 ◽  
Vol 27 (1) ◽  
pp. 28-38 ◽  
Author(s):  
Rosa Cortese ◽  
Carmen Tur ◽  
Ferran Prados ◽  
Torben Schneider ◽  
Baris Kanber ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Brain Mri ◽  
Progressive Multiple Sclerosis ◽  
Cervical Cord ◽  
Primary Progressive Multiple Sclerosis ◽  
Disability Progression ◽  
Mixed Effect ◽  
Primary Progressive ◽  
Over Time

Background: Pathology in the spinal cord of patients with primary progressive multiple sclerosis (PPMS) contributes to disability progression. We previously reported abnormal Q-space imaging (QSI)-derived indices in the spinal cord at baseline in patients with early PPMS, suggesting early neurodegeneration. Objective: The aim was to investigate whether changes in spinal cord QSI over 3 years in the same cohort are associated with disability progression and if baseline QSI metrics predict clinical outcome. Methods: Twenty-three PPMS patients and 23 healthy controls recruited at baseline were invited for follow-up cervical cord 3T magnetic resonance imaging (MRI) and clinical assessment after 1 year and 3 years. Cord cross-sectional area (CSA) and QSI measures were obtained, together with standard brain MRI measures. Mixed-effect models assessed MRI changes over time and their association with clinical changes. Linear regression identified baseline MRI indices associated with disability at 3 years. Results: Over time, patients deteriorated clinically and showed an increase in cord QSI indices of perpendicular diffusivity that was associated with disability worsening, independently of the decrease in CSA. Higher perpendicular diffusivity and lower CSA at baseline predicted worse disability at 3 years. Conclusion: Increasing spinal cord perpendicular diffusivity may indicate ongoing neurodegeneration, which underpins disability progression in PPMS, independently of the development of spinal cord atrophy.

At the heart of primary progressive multiple sclerosis: three cases with diffuse MRI abnormalities only

2008 ◽  
Vol 14 (3) ◽  
pp. 428-430 ◽  
Author(s):  
JNP Zwemmer ◽  
JCJ Bot ◽  
B. Jelles ◽  
F. Barkhof ◽  
CH Polman
Keyword(s):  
Multiple Sclerosis ◽  
Clinical Course ◽  
Progressive Multiple Sclerosis ◽  
Primary Progressive Multiple Sclerosis ◽  
Resonance Imaging ◽  
Focal Lesions ◽  
Primary Progressive ◽  
Magnetic Resonance Imaging Mri ◽  
Brain And Spinal Cord ◽  
Made In

We present three patients with a clinical course and cerebrospinal fluid findings consistent with a diagnosis of primary progressive multiple sclerosis (PPMS). Extensive and repeated magnetic resonance imaging (MRI) examinations showed only diffuse abnormality in brain and spinal cord, but no focal lesions. We propose that these cases represent the most pure form of PPMS, even though according to currently applied criteria this diagnosis can not be made in the absence of focal lesions on MRI. Multiple Sclerosis 2008; 14: 428—430. http://msj.sagepub.com

Preferential spinal cord volume loss in primary progressive multiple sclerosis

2018 ◽  
Vol 25 (7) ◽  
pp. 947-957 ◽  
Author(s):  
Charidimos Tsagkas ◽  
Stefano Magon ◽  
Laura Gaetano ◽  
Simon Pezold ◽  
Yvonne Naegelin ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Clinical Outcomes ◽  
Brain Mri ◽  
Progressive Multiple Sclerosis ◽  
Lesion Volume ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Over Time ◽  
Spinal Cord Volume

Background: Little is known on longer term changes of spinal cord volume (SCV) in primary progressive multiple sclerosis (PPMS). Objective: Longitudinal evaluation of SCV loss in PPMS and its correlation to clinical outcomes, compared to relapse-onset multiple sclerosis (MS) subtypes. Methods: A total of 60 MS age-, sex- and disease duration-matched patients (12 PPMS, each 24 relapsing-remitting (RRMS) and secondary progressive MS (SPMS)) were analysed annually over 6 years of follow-up. The upper cervical SCV was measured on 3D T1-weighted magnetization-prepared rapid gradient-echo (MPRAGE) images using a semi-automatic software (CORDIAL), along with the total brain volume (TBV), brain T2 lesion volume (T2LV) and Expanded Disability Status Scale (EDSS). Results: PPMS showed faster SCV loss over time than RRMS ( p < 0.01) and by trend ( p = 0.066) compared with SPMS. In contrast to relapse-onset MS, in PPMS SCV loss progressed independent of TBV and T2LV changes. Moreover, in PPMS, SCV was the only magnetic resonance imaging (MRI) measurement associated with EDSS increase over time ( p < 0.01), as opposed to RRMS and SPMS. Conclusion: SCV loss is a strong predictor of clinical outcomes in PPMS and has shown to be faster and independent of brain MRI metrics compared to relapse-onset MS.

A proposed modification to the McDonald 2010 criteria for the diagnosis of primary progressive multiple sclerosis

2012 ◽  
Vol 19 (8) ◽  
pp. 1095-1100 ◽  
Author(s):  
SB Kelly ◽  
K Kinsella ◽  
M Duggan ◽  
N Tubridy ◽  
C McGuigan ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Diagnostic Criteria ◽  
Progressive Multiple Sclerosis ◽  
Diagnostic Sensitivity ◽  
Oligoclonal Bands ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Mcdonald Criteria ◽  
The Brain

Background: The diagnostic criteria for primary–progressive multiple sclerosis (PPMS) have undergone revision over the last 20 years. Cerebrospinal fluid oligoclonal bands (CSFOBs) have received less emphasis in recent revisions of the McDonald criteria. The aim of this study was to examine the sensitivity of the diagnostic criteria for PPMS with particular reference to spinal cord criteria and examine the utility of CSFOBs in a cohort of PPMS patients. Methods: All new PPMS diagnoses between 1990 and 2011 were identified. Baseline clinical details and paraclinical evaluations including MRI of the brain, spinal cord, CSF and visually evoked responses (VERs) were assessed. The proportion of patients who met the requirements for diagnosis of PPMS on the basis of Thompson’s and the McDonald Criteria (2001, 2005, 2010) were determined. Results: There were 88/95 PPMS patients who had at least two diagnostic investigations. The sensitivity of Thompson’s and the McDonald 2001 criteria was 64%; the McDonald 2010 revisions gave the highest sensitivity (77%); the McDonald 2005 criteria had intermediate sensitivity (74%). The combination of CSFOBs and MRI of the brain yielded the greatest number of patients demonstrating dissemination in space (DIS) on only two investigations. VERs did not aid diagnosis. Reducing requirements for the number of spinal cord lesions (symptomatic or not) to one increased diagnostic sensitivity to 84%. Conclusion: An alternative criterion requiring two of: i) MRI of the brain with one or more lesions in two of three regions typical for demyelination; ii) the presence of one T2-weighted spinal cord plaque (typical for demyelination); iii) CSFOBs; would increase the diagnostic sensitivity for PPMS.

scholarly journals P.052 Late-onset adrenoleukodystrophy mimicking primary progressive multiple sclerosis

2016 ◽  
Vol 43 (S2) ◽  
pp. S33-S33
Author(s):  
AJ Schabas ◽  
A Sayao
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Phenotypic Variability ◽  
Progressive Multiple Sclerosis ◽  
The Body ◽  
Primary Progressive Multiple Sclerosis ◽  
Primary Progressive ◽  
Progressive Myelopathy ◽  
First Case ◽  
History Of

Background: Adrenoleukodystrophy (ALD) is a peroxisomal disorder that leads to the accumulation of very long chain fatty acids in the body. Younger males typically present with a catastrophic cerebral demyelinating disease, while adult males tend to develop a progressive myelopathy and neuropathy. Methods: Case presentations and literature review. Results:Case1: A 58-year-old male with a three-year history of unsteady gait. His MRI showed multiple T2-hyperintensities most prominently in the posterior corpus collosum (which progressed over time) as well as spinal cord atrophy. Primary progressive multiple sclerosis (PPMS) was suspected. Case 2: The patient’s bother, a 49-year-old, had a ten-year history of difficulty walking. MRI findings included a single large T2 hyper-intensity spanning the anterior aspect of the corpus collosum and an atrophic spinal cord. Given the family history, both brothers were investigated for and diagnosed with ALD. Conclusions: Both cases are of males presenting with a progressive myelopathy in middle age. In the first case, the history, physical exam, and imaging findings were most consistent with PPMS. However, the second case was less typical for MS prompting further investigations. These cases highlight the need to have a broad differential when confronted with atypical cases of MS and reminds the clinician of the phenotypic variability of ADL.

scholarly journals Spinal cord atrophy in a primary progressive multiple sclerosis trial: Improved sample size using GBSI

2020 ◽  
Vol 28 ◽  
pp. 102418 ◽  
Author(s):  
Marcello Moccia ◽  
Nicola Valsecchi ◽  
Olga Ciccarelli ◽  
Ronald Van Schijndel ◽  
Frederik Barkhof ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Sample Size ◽  
Progressive Multiple Sclerosis ◽  
Primary Progressive Multiple Sclerosis ◽  
Spinal Cord Atrophy ◽  
Primary Progressive
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