Evidence map›Paper›PMID 40013114›Full record

ArticleFrontiers in pediatrics2025

Treatment efficacy for infantile epileptic spasms syndrome in children with trisomy 21.

Henry Chen, Adam L Numis, Renée A Shellhaas, John R Mytinger, Debopam Samanta, Rani K Singh, Shaun A Hussain, Danielle Takacs, Kelly G Knupp, Li-Rong Shao and 1 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Frontiers in pediatrics, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
1citing 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

1 citing paper in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
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

11 authors.

Henry Chen *Department of Neurology and Weill Institute for Neuroscience, University of California San Francisco, San Francisco, CA, United States.
Adam L Numis *Department of Neurology and Weill Institute for Neuroscience, University of California San Francisco, San Francisco, CA, United States.
Renée A ShellhaasDepartment of Neurology, Washington University at St. Louis, St. Louis, MO, United States.
John R MytingerDivision of Pediatric Neurology, Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, United States.
Debopam SamantaDepartment of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR, United States.
Rani K SinghDepartment of Pediatrics, Atrium Health-Levine Children's Hospital, Charlotte, NC, United States.
Shaun A HussainDivision of Pediatric Neurology, Department of Pediatrics, University of California, Los Angeles, CA, United States.
Danielle TakacsDepartment of Pediatric Neurology and Developmental Neuroscience, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, United States.
Kelly G KnuppDepartments of Pediatrics and Neurology, University of Colorado Anschutz Medical Campus, Aurora, CO, United States.
Li-Rong ShaoDivision of Pediatric Neurology, Department of Neurology, Johns Hopkins University, Baltimore, MD, United States.
Carl E StafstromDivision of Pediatric Neurology, Department of Neurology, Johns Hopkins University, Baltimore, MD, United States.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Infantile Epileptic Spasms Syndrome (IESS) is the most common epilepsy syndrome in children with trisomy 21. First-line standard treatments for IESS include adrenocorticotropic hormone (ACTH), oral corticosteroids, and vigabatrin. Among children with trisomy 21 and IESS, treatment with ACTH or oral corticosteroids may yield higher response rates compared with vigabatrin. However, supporting data are largely from single-center, retrospective cohort studies. Methods: Leveraging the multi-center, prospective National Infantile Spasms Consortium (NISC) database, we evaluated the efficacy of first-line (standard) treatments for IESS in children with trisomy 21. We assessed clinical spasms remission at two weeks, clinical spasms remission at three months, and improvement of EEG (resolution of hypsarrhythmia) three months after initiation of treatment. Results: Thirty four of 644 (5.3%) children with IESS were diagnosed with trisomy 21. In all children with trisomy 21, epileptic spasms was their presenting seizure type. Twenty of 34 (59%) children were initially treated with ACTH, nine (26%) with oral corticosteroids, and five (15%) with vigabatrin. Baseline demographics did not vary among treatment groups. The overall clinical remission rate after two weeks of treatment was 53% including 13 of 20 (65%) receiving ACTH, three of nine (33%) receiving oral corticosteroids, and two of five (40%) receiving vigabatrin ( Conclusions: In our cohort, epileptic spasms were the first presenting seizure type in all children with trisomy 21. Among first-line standard treatment options, ACTH may have superior efficacy for clinical and electrographic outcomes for IESS in children with trisomy 21.

Indexed as

anti-seizure medicationsDown syndromehypsarrhythmiainfantile epileptic spasms syndromeinfantile spasmstrisomy 21

Identifiers

PMID40013114
PMCPMC11860949

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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 OpenQuestion graph.