scholarly journals Pediatric Multiple Sclerosis Severity Score in a large US cohort

Neurology ◽  
2020 ◽  
Vol 95 (13) ◽  
pp. e1844-e1853
Author(s):  
Jonathan D. Santoro ◽  
Michael Waltz ◽  
Greg Aaen ◽  
Anita Belman ◽  
Leslie Benson ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Retrospective Analysis ◽  
Disease Severity ◽  
Severity Score ◽  
Functional Score ◽  
Published Data ◽  
Pediatric Multiple Sclerosis ◽  
Severity Scores ◽  
The Us ◽  
Duration Of Disease

ObjectiveTo characterize disease severity and distribution of disability in pediatric-onset multiple sclerosis (POMS) and to develop an optimized modeling scale for measuring disability, we performed a multicenter retrospective analysis of disability scores in 873 persons with POMS over time and compared this to previously published data in adults with multiple sclerosis (MS).MethodsThis was a retrospective analysis of prospectively collected data collected from 12 centers of the US Network of Pediatric MS Centers. Patients were stratified by the number of years from first symptoms of MS to Expanded Disability Status Scale (EDSS) assessment and an MS severity score (Pediatric Multiple Sclerosis Severity Score [Ped-MSSS]) was calculated per criteria developed by Roxburgh et al. in 2005.ResultsIn total, 873 patients were evaluated. In our cohort, 52%, 19.4%, and 1.5% of all patients at any time point reached an EDSS of 2.0, 3.0, and 6.0. Comparison of our Ped-MSSS scores and previously published adult Multiple Sclerosis Severity Scores (MSSS) showed slower progression of Ped-MSSS with increasing gaps between higher EDSS score and years after diagnosis. Decile scores in our POMS cohort for EDSS of 2.0, 3.0, and 6.0 were 8.00/9.46/9.94, 7.86/9.39/9.91, and 7.32/9.01/9.86 at 2, 5, and 10 years, respectively. Notable predictors of disease progression in both EDSS and Ped-MSSS models were ever having a motor relapse and EDSS at year 1. Symbol Digit Modalities Test (SDMT) scores were inversely correlated with duration of disease activity and cerebral functional score.ConclusionsPersons with POMS exhibit lower EDSS scores compared to persons with adult-onset MS. Use of a Ped-MSSS model may provide an alternative to EDSS scoring in clinical assessment of disease severity and disability accrual.

scholarly journals Use of newer disease-modifying therapies in pediatric multiple sclerosis in the US

Neurology ◽  
2018 ◽  
Vol 91 (19) ◽  
pp. e1778-e1787 ◽  
Author(s):  
Kristen M. Krysko ◽  
Jennifer Graves ◽  
Mary Rensel ◽  
Bianca Weinstock-Guttman ◽  
Gregory Aaen ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Side Effect ◽  
Pediatric Patients ◽  
Dimethyl Fumarate ◽  
Short Term ◽  
Pediatric Multiple Sclerosis ◽  
Disease Modifying Therapies ◽  
The Us ◽  
Disease Modifying

ObjectiveTo characterize the use and safety of newer disease-modifying therapies (DMTs) in children with multiple sclerosis (MS) and clinically isolated syndrome (CIS) treated under 18 years of age.MethodsThis is a cohort study including children with MS or CIS followed at 12 outpatient practices participating in the US Network of Pediatric MS Centers. DMT use, including duration, dose, and side effects, was analyzed. Newer DMTs were defined as agents receiving Food and Drug Administration approval or with increased use in adult MS after 2005.ResultsAs of July 2017, 1,019 pediatric patients with MS (n = 748) or CIS (n = 271) were enrolled (65% female, mean onset 13.0 ± 3.9 years, mean follow-up 3.5 ± 3.1 years, median 1.6 visits per year). Of these, 78% (n = 587) with MS and 11% (n = 31) with CIS received DMT before 18 years of age. This consisted of at least one newer DMT in 42%, including dimethyl fumarate (n = 102), natalizumab (n = 101), rituximab (n = 57), fingolimod (n = 37), daclizumab (n = 5), and teriflunomide (n = 3). Among 17%, the initial DMT prescribed was a newer agent (36 dimethyl fumarate, 30 natalizumab, 22 rituximab, 14 fingolimod, 2 teriflunomide). Over the last 10 years, the use of newer agents has increased, particularly in those ≥12 years and to lesser extent in those <12 years. The short-term side effect profiles of newer DMTs did not differ from those reported in adults.ConclusionNewer DMTs are often used in pediatric MS, and have similar short-term safety, tolerability, and side effect profiles as in adults. These findings may help inform pediatric MS management.

scholarly journals The US Network of Pediatric Multiple Sclerosis Centers

2014 ◽  
Vol 30 (10) ◽  
pp. 1381-1387 ◽  
Author(s):  
T. Charles Casper ◽  
John W. Rose ◽  
Shelly Roalstad ◽  
Emmanuelle Waubant ◽  
Gregory Aaen ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Pediatric Multiple Sclerosis ◽  
The Us

Is intralesional cidofovir worthwhile in juvenile recurrent respiratory papillomatosis?

2006 ◽  
Vol 120 (7) ◽  
pp. 561-565 ◽  
Author(s):  
P Sheahan ◽  
S Sexton ◽  
J D Russell
Keyword(s):  
Disease Severity ◽  
Severity Score ◽  
Prospective Observational Study ◽  
Recurrent Respiratory Papillomatosis ◽  
Prolonged Treatment ◽  
Disease Remission ◽  
Treatment Regimes ◽  
Severity Scores ◽  
Surgical Debulking ◽  
Reduce Disease Severity

Objective: To investigate the efficacy of intralesional cidofovir in the treatment of recurrent respiratory papillomatosis (RRP) in children.Methods: Prospective observational study of four consecutive children with RRP treated at an academic tertiary children's hospital. Laryngo-bronchoscopy was performed at three- to five-weekly intervals. Photodocumentation was obtained and disease severity assessed using an anatomical RRP severity score. Surgical debulking of large papillomas was then performed, and cidofovir (5 mg/ml) injected into any remaining papillomas as well as submucosally at the sites of resected papillomas. The efficacy of cidofovir was assessed by the change in papilloma severity score over the course of the treatment.Results: Complete disease remission was obtained in one patient, with a partial response seen in two others. One patient showed no significant response. The greatest beneficial effect was seen after the fourth cidofovir injection; however, two patients demonstrated a deterioration in severity scores after treatment was withheld at this point. Both responded well to further cidofovir injections. However, a clear plateau in the response to cidofovir was seen in all patients by the eighth injection.Conclusion: Intralesional cidofovir may help control papilloma regrowth and reduce disease severity in many children with RRP. In most cases, cidofovir would appear to be less efficacious in causing disease eradication. There appears to be little evidence to support prolonged treatment regimes (i.e. more than eight treatments).

Multiple Sclerosis Severity Score: Using disability and disease duration to rate disease severity

Neurology ◽  
2005 ◽  
Vol 64 (7) ◽  
pp. 1144-1151 ◽  
Author(s):  
R. H.S.R. Roxburgh ◽  
S. R. Seaman ◽  
T. Masterman ◽  
A. E. Hensiek ◽  
S. J. Sawcer ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Disease Severity ◽  
Severity Score ◽  
Disease Duration

scholarly journals Clinical Decision Support Tool and Rapid Point-of-Care Platform for Determining Disease Severity in Patients with COVID-19

2020 ◽  
Author(s):  
Michael P. McRae ◽  
Glennon W. Simmons ◽  
Nicolaos J. Christodoulides ◽  
Zhibing Lu ◽  
Stella K. Kang ◽  
...  
Keyword(s):  
Decision Support ◽  
Disease Severity ◽  
Clinical Decision Support ◽  
Severity Score ◽  
Troponin I ◽  
Point Of Care ◽  
Clinical Decision ◽  
Adverse Outcomes ◽  
Number Of Patients ◽  
Severity Scores

AbstractSARS-CoV-2 is the virus that causes coronavirus disease (COVID-19) which has reached pandemic levels resulting in significant morbidity and mortality affecting every inhabited continent. The large number of patients requiring intensive care threatens to overwhelm healthcare systems globally. Likewise, there is a compelling need for a COVID-19 disease severity test to prioritize care and resources for patients at elevated risk of mortality. Here, an integrated point-of-care COVID-19 Severity Score and clinical decision support system is presented using biomarker measurements of C-reactive protein (CRP), N-terminus pro B type natriuretic peptide (NT-proBNP), myoglobin (MYO), D-dimer, procalcitonin (PCT), creatine kinase–myocardial band (CK-MB), and cardiac troponin I (cTnI). The COVID-19 Severity Score combines multiplex biomarker measurements and risk factors in a statistical learning algorithm to predict mortality. The COVID-19 Severity Score was trained and evaluated using data from 160 hospitalized COVID-19 patients from Wuhan, China. Our analysis finds that COVID-19 Severity Scores were significantly higher for the group that died versus the group that was discharged with median (interquartile range) scores of 59 (40–83) and 9 (6–17), respectively, and area under the curve of 0.94 (95% CI 0.89– 0.99). These promising initial models pave the way for a point-of-care COVID-19 Severity Score system to impact patient care after further validation with externally collected clinical data. Clinical decision support tools for COVID-19 have strong potential to empower healthcare providers to save lives by prioritizing critical care in patients at high risk for adverse outcomes.

Ring-enhancement in multiple sclerosis: marker of disease severity

2001 ◽  
Vol 7 (3) ◽  
pp. 167-167 ◽  
Author(s):  
K Morgen ◽  
N O Jeffries ◽  
R Stone ◽  
R Martin ◽  
N D Richert ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Disease Severity ◽  
Multiple Logistic Regression Analysis ◽  
Baseline Period ◽  
Pathological Process ◽  
Magnetic Resonance Images ◽  
Conventional Mri ◽  
Potential Association ◽  
Relapsing Remitting ◽  
Duration Of Disease

Correlations between conventional MRI measures of disease activity and clinical disability in multiple sclerosis (MS) have been disappointing. Because ring-enhancing lesions may reflect a more destructive pathology, we tested their potential association with disease severity. We evaluated active lesions with regard to their enhancement pattern on serial magnetic resonance images in a cohort of 28 patients with relapsing-remitting MS. The percentage of ring-enhancing lesions correlated with EDSS, T2 lesion load and duration of disease and predicted the occurrence of relapses during the baseline period of observation as well as after 3 years of follow-up in multiple logistic regression analysis. The findings suggest that the pathological process reflected by ring-enhancing lesions may contribute to more severe clinical disease.

scholarly journals Longitudinal evaluation of cognitive functioning in pediatric multiple sclerosis: report from the US Pediatric Multiple Sclerosis Network

2014 ◽  
Vol 20 (11) ◽  
pp. 1502-1510 ◽  
Author(s):  
LE Charvet ◽  
EH O’Donnell ◽  
AL Belman ◽  
T Chitnis ◽  
JM Ness ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Cognitive Functioning ◽  
Verbal Memory ◽  
Neuropsychological Testing ◽  
Visuomotor Integration ◽  
Pediatric Multiple Sclerosis ◽  
Age Related ◽  
The Us ◽  
Over Time

Background: Approximately one-third of those with pediatric-onset multiple sclerosis (MS) experience cognitive impairment. Less is known concerning their change in cognitive functioning over time. Objective: Changes in cognitive function over time were measured in the largest pediatric cohort to date through the US Network of Pediatric MS Centers. Methods: A total of 67 individuals with pediatric MS ( n=62) or clinically isolated syndrome (CIS, n=5), ranging from 8–17 years of age (mean age±standard deviation (SD)=14.37±2.02) completed initial and follow-up neuropsychological testing after an average of 1.64±0.63 years apart. The nine tests administered measure general intellect, attention and working memory, verbal memory, visuomotor integration, language, and executive functioning. Results: Rate of impairment (having one-third or more scores in the impaired range) was 37% at baseline and 33% at follow-up. Tests commonly impaired were measures of visuomotor integration, speeded processing, and attention. Most tested did not decline over two years. There was no clear pattern of change on any specific measure. Conclusion: Findings suggest that, over short timeframes, stable or even improved performances on measures of cognitive ability can occur. Pediatric MS may instead prevent expected age-related cognitive gains.

Analysis of multiple candidate genes in association with phenotypes of multiple sclerosis

2010 ◽  
Vol 16 (6) ◽  
pp. 652-659 ◽  
Author(s):  
Madeleine H Sombekke ◽  
David Arteta ◽  
Mark A van de Wiel ◽  
J Bart A Crusius ◽  
Diego Tejedor ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Single Nucleotide Polymorphisms ◽  
Disease Severity ◽  
Clinical Data ◽  
Severity Score ◽  
Interleukin 2 ◽  
Dna Chip ◽  
Nucleotide Polymorphisms ◽  
Single Nucleotide ◽  
Clinical Variables

Multiple sclerosis is a heterogeneous neurological disease with varying degrees of severity. The common hypothesis is that susceptibility to multiple sclerosis and its phenotype are caused by a combination of environmental and genetic factors. The genetic part exerts its effect through several genes, each having modest effects. We evaluated whether disease severity could be predicted by a model based on clinical data and data from a DNA chip. The DNA chip was designed containing several single nucleotide polymorphisms in 44 genes, previously described to be associated with multiple sclerosis. A total of 605 patients with multiple sclerosis were included in this analysis, using gender, onset type and age at onset as clinical covariates. We correlated 80 single nucleotide polymorphisms to the degree of disease severity using the following three outcome measures: linear Multiple Sclerosis Severity Score, dichotomous Multiple Sclerosis Severity Score (using a cut-off point of 2.5) and time to reach Expanded Disability Status Scale score 6. Sixty-nine single nucleotide polymorphisms were included in the analysis. No individual single nucleotide polymorphism showed a significant association; however, a combination of single nucleotide polymorphisms significantly improved the prediction of disease severity in addition to the clinical variables. In all three models the Interleukin 2 gene was included, confirming a previously reported modest effect on disease severity. The highest power was obtained using the dichotomized Multiple Sclerosis Severity Score as outcome. Several single nucleotide polymorphisms showed their added predictive value over the clinical data in the predictive models. These results support our hypothesis that disease severity is determined by clinical variables and genetic influences (through several genes with small effects) in concert.

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