Evidence mapPaperPMID 40368588Full record

ArticleAnnals of clinical and translational neurology2025

Myostatin Levels in SMA Following Disease-Modifying Treatments: A Multi-Center Study.

Fiorella Piemonte, Sara Petrillo, Anna Capasso, Giorgia Coratti, Adele D'Amico, Michela Catteruccia, Maria Carmela Pera, Concetta Palermo, Marika Pane, Emanuela Abiusi and 14 more

Abstract readMulticenter Study
In one paragraph

Article in Annals of clinical and translational neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed, 1 pooled it
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 synthesis or guideline pooled it.

  1. Pooled it
  2. Review
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

24 authors.

Fiorella PiemonteDepartment of Neurosciences, Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Sara PetrilloDepartment of Neurosciences, Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Anna CapassoPediatric Neurology Unit, Catholic University, Rome, Italy.
Giorgia CorattiPediatric Neurology Unit, Catholic University, Rome, Italy.
Adele D'AmicoDepartment of Neurosciences, Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.ORCID 0000-0003-2438-2624
Michela CatterucciaDepartment of Neurosciences, Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Maria Carmela PeraPediatric Neurology Unit, Catholic University, Rome, Italy.ORCID 0000-0001-6777-1721
Concetta PalermoPediatric Neurology Unit, Catholic University, Rome, Italy.
Marika PanePediatric Neurology Unit, Catholic University, Rome, Italy.
Emanuela AbiusiSection of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Rome, Italy.
Gianpaolo CicalaPediatric Neurology Unit, Catholic University, Rome, Italy.
Marianna VillaPediatric Neurology Unit, Catholic University, Rome, Italy.
Chiara BravettiPediatric Neurology Unit, Catholic University, Rome, Italy.
Chiara ArpaiaPediatric Neurology Unit, Catholic University, Rome, Italy.
Agnese NovelliSection of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Rome, Italy.
Salvatore FalquiSection of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Rome, Italy.
Stefania FioriSection of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Rome, Italy.
Giulia NapoliDepartment of Basic Biotechnological Sciences, Intensive Care and Perioperative Clinics, Catholic University of Sacred Heart, Rome, Italy.
Silvia BaroniDepartment of Basic Biotechnological Sciences, Intensive Care and Perioperative Clinics, Catholic University of Sacred Heart, Rome, Italy.
Francesco Danilo TizianoSection of Genomic Medicine, Department of Life Sciences and Public Health, Catholic University of Sacred Heart, Rome, Italy.ORCID 0000-0002-5545-6158
Enrico BertiniDepartment of Neurosciences, Unit of Neuromuscular and Neurodegenerative Disorders, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Giacomo ComiSSD Neuromuscular and Rare Diseases, Department of Neurosciences and Mental Health, Fondazione IRCCS ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
Stefania CortiSSD Neuromuscular and Rare Diseases, Department of Neurosciences and Mental Health, Fondazione IRCCS ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.ORCID 0000-0001-5425-969X
Eugenio MercuriPediatric Neurology Unit, Catholic University, Rome, Italy.

Funding

Ministero della Salute GR-2021-12374579Ministero della Salute RF-2019-12370334
6 · The paper itself

Abstract

objectiveThis study investigated myostatin levels in SMA patients receiving disease-modifying therapies (DMTs) to understand their relationship with treatment duration and functional status.

methodsOur study includes both cross-sectional and longitudinal analyses of myostatin levels in treated SMA patients. The longitudinal cohort included 46 treatment-naive patients assessed at baseline and 12 months post-treatment. Myostatin levels were measured using ELISA. Age-matched controls (n = 89) were included for comparison. The cross-sectional study included 128 patients with variable durations of treatment (from 0.4 to 7.2 years). In both cohorts, myostatin levels were correlated with SMA type, functional status, and clinical outcomes.

resultsBaseline myostatin levels were significantly lower than controls (p < 0.001), except during the neonatal period in presymptomatic patients. After 12 months of treatment, there were no significant changes compared to baseline levels (p = 0.1652). The only substantial changes were observed in presymptomatic neonates, who showed a reduction of myostatin despite treatment intervention. There was a significant correlation between myostatin levels, functional status, and SMA type both in the cross-sectional and longitudinal groups.

interpretationThis study demonstrates lower myostatin levels in SMA patients compared to controls. The association between myostatin levels, functional status, and SMA type suggests its possible role as a disease severity biomarker. The utility of myostatin as a biomarker for DMT response remains controversial; while we observed no significant increase in myostatin levels following treatment, we also did not observe the progressive reduction previously reported in untreated patients.

Indexed as

Muscular Atrophy, SpinalMyostatinChildChild, PreschoolCross-Sectional StudiesFemaleHumansInfantInfant, NewbornLongitudinal StudiesMaleMSTN protein, humanMyostatinbiomarkersdisease‐modifying therapiesmyostatinneuromuscular disordersspinal muscular atrophytreatment response

Identifiers

PMID40368588
PMCPMC12257122

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.