Evidence mapPaperPMID 40577272Full record

ArticlePloS one2025

Predicting trajectories of the north star ambulatory assessment total score in Duchenne muscular dystrophy.

Francesco Muntoni, James Signorovitch, Nathalie Goemans, Adnan Y Manzur, Nicolae Done, Gautam Sajeev, Jiayang Li, Hanane Akbarnejad, Aarushi Sharma, Susan J Ward and 11 more

Abstract read
In one paragraph

Article in PloS one, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Article
  2. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

21 authors.

Francesco MuntoniDubowitz Neuromuscular Centre, NIHR Great Ormond Street Hospital Biomedical Research Centre, Great Ormond Street Institute of Child Health, University College London, & Great Ormond Street Hospital Trust, London, United Kingdom.
James SignorovitchAnalysis Group, Boston, Massachusetts, United States of America.ORCID https://orcid.org/0000-0002-4067-8962
Nathalie GoemansChild Neurology, University Hospitals Leuven, Leuven, Belgium.
Adnan Y ManzurDubowitz Neuromuscular Centre, NIHR Great Ormond Street Hospital Biomedical Research Centre, Great Ormond Street Institute of Child Health, University College London, & Great Ormond Street Hospital Trust, London, United Kingdom.
Nicolae DoneAnalysis Group, Boston, Massachusetts, United States of America.
Gautam SajeevAnalysis Group, Boston, Massachusetts, United States of America.
Jiayang LiAnalysis Group, Boston, Massachusetts, United States of America.
Hanane AkbarnejadAnalysis Group, Boston, Massachusetts, United States of America.
Aarushi SharmaAnalysis Group, Boston, Massachusetts, United States of America.
Susan J WardThe collaborative Trajectory Analysis Project, Cambridge, Massachusetts, United States of America.ORCID https://orcid.org/0000-0002-5729-5263
Erik H NiksDepartment of Neurology, Leiden University Medical Center, Leiden, Belgium.
Laurant ServaisDivision of Child Neurology, Department of Pediatrics, Centre de Référence des Maladies Neuromusculaires, University Hospital Liège and University of Liège, Liège, Belgium & Department of Paediatrics, MDUK Oxford Neuromuscular Centre & NIHR Oxford Biomedical Research Centre, University of Oxford, Oxford, United Kingdom.
Eugenio MercuriPaediatric Neurology, Catholic University, Rome, Italy.
Michela GuglieriThe John Walton Muscular Dystrophy Research Centre, NIHR Newcastle Biomedical Research Centre, Newcastle University and Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom.
Volker StraubThe John Walton Muscular Dystrophy Research Centre, NIHR Newcastle Biomedical Research Centre, Newcastle University and Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom.
Imelda de GrootAcademic Medical Center Amsterdam, Rehabilitation Center De Trappenberg in Huizen and Radboud University Medical Center, Nijmegen, Netherlands.
Deborah RidoutPopulation Policy and Practice Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, London, United Kingdom.ORCID https://orcid.org/0000-0003-1336-6450
PRO-DMD-01 study investigators
Association Française contre les Myopathies
NorthStar Clinical Network
Craig McDonaldDepartment of Physical Medicine and RehabilitationPediatrics, University of California Davis, Sacramento, California, United States of America.ORCID https://orcid.org/0000-0002-8779-3220

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

The North Star Ambulatory Assessment (NSAA) is a widely used functional endpoint in drug development for ambulatory patients with Duchenne muscular dystrophy (DMD). Accurately predicting NSAA total score trajectories is important for designing randomized trials for novel therapies in DMD and for contextualizing outcomes, especially over longer-term follow-up (>18 months) when placebo-controlled studies are infeasible. We developed a prognostic model for NSAA total score trajectories over at most 5 years of follow-up for patients with DMD aged 4 to <16 years who were initially ambulatory and receiving corticosteroids but no other disease-modifying therapies. The model was based on longitudinal data from four natural history databases: UZ Leuven, PRO-DMD-01 (provided by CureDuchenne), the North Star Clinical Network, and iMDEX. Candidate predictors included age, height, weight, body mass index, steroid type and regime, NSAA total score, rise from floor velocity, and 10-meter walk/run velocity, as well as DMD genotype class, index year, and data source. Among N = 416 patients at baseline, mean age was 8.2 years, mean NSAA total score was 24, and 61% were receiving prednisone and 39% deflazacort, with the majority having been treated with daily corticosteroid regimens (69%) relative to other regimens (31%). Patients had an average of four NSAA assessments post-baseline during a median follow-up of 2.6 years (inter-quartile range 1.9 to 3.6 years). The best-fitting model in the full study sample explained 39% of the variation in NSAA total score changes, with prediction errors of ±3.6, 5.1, 5.9, 7.5, 9.5 NSAA units during follow-up years 1-5, respectively. The most important predictors were baseline age, NSAA, rise from floor velocity, and 10-meter walk/run velocity. In conclusion, trajectories of ambulatory motor function in DMD, as measured by the NSAA total score, can be well-predicted using readily available baseline characteristics. We discuss applications of these predictions to DMD drug development.

Indexed as

Muscular Dystrophy, DuchenneAdolescentAdrenal Cortex HormonesChildChild, PreschoolHumansMalePrognosisWalkingAdrenal Cortex Hormones

Identifiers

PMID40577272
PMCPMC12204569

What Socratic holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.