Literature DB >> 23909763

Detecting meaningful change using the North Star Ambulatory Assessment in Duchenne muscular dystrophy.

Anna G Mayhew1, Stefan J Cano, Elaine Scott, Michelle Eagle, Kate Bushby, Adnan Manzur, Francesco Muntoni.   

Abstract

AIM: Clinician-reported outcome instruments such as the North Star Ambulatory Assessment (NSAA) need to be able to detect clinically important change to be suitable for clinical trials. However, in Duchenne muscular dystrophy (DMD), identifying changes in function is not straightforward. In this study, we use Rasch-transformed data to examine the responsiveness and minimal important difference (MID) of the NSAA in males with DMD receiving different corticosteroid regimes.
METHOD: NSAA data were examined from 198 males (mean age at assessment was 8 y 6 mo [SD 2 y 6 mo] range 4 y-18 y; 805 assessments). Responsiveness was assessed using mean score changes (using Rasch-transformed data) between adjacent pairs of age groups, pairwise squared t-values from paired samples t-tests, and an effect size calculation. The MID was assessed using the effect size calculation and 0.5 standard deviation (SD) of mean score differences.
RESULTS: Our findings revealed a difference in change scores over time between the two corticosteroid regimes. Mean NSAA person estimates were higher in the daily prednisolone group. The mean MID (0.5 SD) was 8.8 and 6.9 for the daily group and intermittent group respectively.
INTERPRETATION: This study, based on Rasch-transformed NSAA data, provides an initial basis for the interpretation of clinical change in DMD over time and between corticosteroid regimes. Our proposed MIDs can be mapped back to differences in specific item content across the range of the NSAA.
© 2013 Mac Keith Press.

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Year:  2013        PMID: 23909763     DOI: 10.1111/dmcn.12220

Source DB:  PubMed          Journal:  Dev Med Child Neurol        ISSN: 0012-1622            Impact factor:   5.449


  17 in total

Review 1.  Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management.

Authors:  David J Birnkrant; Katharine Bushby; Carla M Bann; Susan D Apkon; Angela Blackwell; David Brumbaugh; Laura E Case; Paula R Clemens; Stasia Hadjiyannakis; Shree Pandya; Natalie Street; Jean Tomezsko; Kathryn R Wagner; Leanne M Ward; David R Weber
Journal:  Lancet Neurol       Date:  2018-02-03       Impact factor: 44.182

2.  Quantitative magnetic resonance imaging measures as biomarkers of disease progression in boys with Duchenne muscular dystrophy: a phase 2 trial of domagrozumab.

Authors:  Sarah P Sherlock; Jeffrey Palmer; Kathryn R Wagner; Hoda Z Abdel-Hamid; Enrico Bertini; Cuixia Tian; Jean K Mah; Anna Kostera-Pruszczyk; Francesco Muntoni; Michela Guglieri; John F Brandsema; Eugenio Mercuri; Russell J Butterfield; Craig M McDonald; Lawrence Charnas; Shannon Marraffino
Journal:  J Neurol       Date:  2022-04-08       Impact factor: 6.682

3.  The NorthStar Ambulatory Assessment in Duchenne muscular dystrophy: considerations for the design of clinical trials.

Authors:  Valeria Ricotti; Deborah A Ridout; Marika Pane; Marion Main; Anna Mayhew; Eugenio Mercuri; Adnan Y Manzur; Francesco Muntoni
Journal:  J Neurol Neurosurg Psychiatry       Date:  2015-03-02       Impact factor: 10.154

4.  Aquatic therapy for boys with Duchenne muscular dystrophy (DMD): an external pilot randomised controlled trial.

Authors:  Daniel Hind; James Parkin; Victoria Whitworth; Saleema Rex; Tracey Young; Lisa Hampson; Jennie Sheehan; Chin Maguire; Hannah Cantrill; Elaine Scott; Heather Epps; Marion Main; Michelle Geary; Heather McMurchie; Lindsey Pallant; Daniel Woods; Jennifer Freeman; Ellen Lee; Michelle Eagle; Tracey Willis; Francesco Muntoni; Peter Baxter
Journal:  Pilot Feasibility Stud       Date:  2017-03-27

Review 5.  Measuring what matters to rare disease patients - reflections on the work by the IRDiRC taskforce on patient-centered outcome measures.

Authors:  Thomas Morel; Stefan J Cano
Journal:  Orphanet J Rare Dis       Date:  2017-11-02       Impact factor: 4.123

6.  Magnetic resonance imaging and spectroscopy assessment of lower extremity skeletal muscles in boys with Duchenne muscular dystrophy: a multicenter cross sectional study.

Authors:  Sean C Forbes; Rebecca J Willcocks; William T Triplett; William D Rooney; Donovan J Lott; Dah-Jyuu Wang; Jim Pollaro; Claudia R Senesac; Michael J Daniels; Richard S Finkel; Barry S Russman; Barry J Byrne; Erika L Finanger; Gihan I Tennekoon; Glenn A Walter; H Lee Sweeney; Krista Vandenborne
Journal:  PLoS One       Date:  2014-09-09       Impact factor: 3.240

7.  Can Quantitative Muscle Strength and Functional Motor Ability Differentiate the Influence of Age and Corticosteroids in Ambulatory Boys with Duchenne Muscular Dystrophy?

Authors:  Cathleen Buckon; Susan Sienko; Anita Bagley; Mitell Sison-Williamson; Eileen Fowler; Loretta Staudt; Kent Heberer; Craig M McDonald; Michael Sussman
Journal:  PLoS Curr       Date:  2016-07-08

8.  Muscular response to the first three months of deflazacort treatment in boys with Duchenne muscular dystrophy.

Authors:  L Jensen; S J Petersson; N O Illum; H C Laugaard-Jacobsen; T Thelle; L H Jørgensen; H D Schrøder
Journal:  J Musculoskelet Neuronal Interact       Date:  2017-06-01       Impact factor: 2.041

9.  Management of motor rehabilitation in individuals with muscular dystrophies. 1st Consensus Conference report from UILDM - Italian Muscular Dystrophy Association (Rome, January 25-26, 2019).

Authors:  Maria Elena Lombardo; Elena Carraro; Cristina Sancricca; Michela Armando; Michela Catteruccia; Elena Mazzone; Giulia Ricci; Ferdinando Salamino; Filippo Maria Santorelli; Massimiliano Filosto
Journal:  Acta Myol       Date:  2021-06-30

10.  A phase 3 randomized placebo-controlled trial of tadalafil for Duchenne muscular dystrophy.

Authors:  Ronald G Victor; H Lee Sweeney; Richard Finkel; Craig M McDonald; Barry Byrne; Michelle Eagle; Nathalie Goemans; Krista Vandenborne; Alberto L Dubrovsky; Haluk Topaloglu; M Carrie Miceli; Pat Furlong; John Landry; Robert Elashoff; David Cox
Journal:  Neurology       Date:  2017-09-29       Impact factor: 9.910

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