Evidence map›Paper›PMID 36382115›Full record

ArticleNeurology. Clinical practice2022

Scoliosis in Spinal Muscular Atrophy Type 1 in the Nusinersen Era.

Fatima Al Amrani, Reshma Amin, Jackie Chiang, Lena Xiao, Jennifer Boyd, Eugenia Law, Elisa Nigro, Lauren Weinstock, Ana Stosic, Hernan D Gonorazky

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PubMed Full text at PMC Publisher
In one paragraph

Article in Neurology. Clinical practice, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed
5.0field-weighted citation impact, top 4% of its field
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

13 citing papers in PubMed, 35 citations in OpenAlex.

  1. Trial
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  3. Scoliosis development in 5q-spinal muscular atrophy under disease modifying therapies.European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society · 2025
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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

10 authors at 3 institutions in 1 country.

Fatima Al AmraniDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Reshma AminDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Jackie ChiangDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Lena XiaoDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Jennifer BoydDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Eugenia LawDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Elisa NigroDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Lauren WeinstockDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Ana StosicDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Hernan D GonorazkyDepartment of Pediatrics (FAA, JB, EL, EN-P, HDG), Division of Neurology, Hospital for Sick Children, University of Toronto; Department of Pediatrics (RA, JC, LX), Division of Respiratory Medicine, Hospital for Sick Children, University of Toronto; Department of Rehabilitation Services (LW), Hospital for Sick Children, University of Toronto; and Genetics and Genome Biology Program (AS), PGCRL, University of Toronto, Canada.
Hospital for Sick Children · CASickKids Foundation · CAUniversity of Toronto · CA

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background and Objectives: The introduction of spinal muscular dystrophy (SMA)-modifying therapies, such as antisense oligonucleotide therapy, has changed the natural history of SMA. Most reports on treatment outcomes have focused on motor scores and respiratory function. The objective of this study is to document the development and progression of scoliosis in patients with SMA1 treated with nusinersen. Methods: A descriptive single-center study was conducted in patients with SMA1 who were treated with nusinersen before 6 months of age. Data were collected on patients who met criteria, including age at the first nusinersen dose, number of nusinersen doses, degree of scoliosis, respiratory parameters, feeding route, and motor scores at baseline and follow-up. The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) was subanalyzed using axial (AxS) and appendicular motor (ApS) scores to evaluate a possible correlation between scoliosis and axial muscle strength. Results: From our cohort, 31 percent (11/35) of patients had a diagnosis of SMA1. Sixty-three percent (7/11) met the inclusion criteria. All patients (7/7) showed initial improvement in their CHOP-INTEND scores in correlation with improvement on the ApS. Despite this, most patients did not show improvement in the AxS. Subsequently, all patients developed scoliosis in the first year of life with Cobb angles that ranged between 18° and 60°. Furthermore, total CHOP-INTEND scores had dropped in 2 patients alongside the development of a Cobb angle of >40°. Discussion: Despite the significant improvement in functional motor assessment in patients with SMA1, there is a progression of significant scoliosis despite treatment. Subsequently, lack or minimal improvement on the axial CHOP-INTEND scores may predict worsening on the total motor scores.

Identifiers

PMID36382115
PMCPMC9647826
OpenAlexW4281389579

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
Read underepoch 390

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 OpenQuestion graph.