Evidence map›Paper›PMID 37138365›Full record

ArticleOrphanet journal of rare diseases2023

Objective measurement of oral function in adults with spinal muscular atrophy.

T Kruse, S Shamai, D Leflerovà, B Wirth, R Heller, N Schloss, H C Lehmann, S Brakemeier, T Hagenacker, B Braumann and 1 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Orphanet journal of rare diseases, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.

0numbers the graph read from it
0cells of the map it votes in
6citing 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

6 citing papers in PubMed.

  1. Oral function tests in spinal muscular atrophy: closing the diagnostic gap in severely affected adult patients : A prospective observational study.Journal of orofacial orthopedics = Fortschritte der Kieferorthopadie : Organ/official journal Deutsche Gesellschaft fur Kieferorthopadie · 2026
    Observational
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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

11 authors.

T KruseDepartment of Orthodontics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Kerpener Str. 32, 50931, Cologne, Germany. teresa.kruse@uk-koeln.de.ORCID 0000-0002-3431-7749
S ShamaiDepartment of Orthodontics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Kerpener Str. 32, 50931, Cologne, Germany.
D LeflerovàDepartment of Orthodontics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Kerpener Str. 32, 50931, Cologne, Germany.
B WirthCenter for Rare Diseases Cologne, University of Cologne, Cologne, Germany.
R HellerCenter for Rare Diseases Cologne, University of Cologne, Cologne, Germany.
N SchlossDepartment of Neurology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Kerpener Str. 62, 50937, Cologne, Germany.
H C LehmannDepartment of Neurology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Kerpener Str. 62, 50937, Cologne, Germany.
S BrakemeierDepartment of Neurology, Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, Essen, Germany.
T HagenackerDepartment of Neurology, Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, Essen, Germany.
B BraumannDepartment of Orthodontics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Kerpener Str. 32, 50931, Cologne, Germany.
G WunderlichCenter for Rare Diseases Cologne, University of Cologne, Cologne, Germany.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundImpairment of bulbar function in adult individuals with spinal muscular atrophy (SMA) usually is not assessed by established motor scores. Measurements of oral function including quantitative muscle and endurance tests are able to detect subtle changes. The aim of this study was to systematically evaluate the measurement of maximum bite force and endurance, maximum tongue pressure and endurance, as well as maximum mouth opening in adult individuals with SMA types 2 and 3.

methodsData from oral function tests in 43 individuals were analyzed. Differences in oral function between individuals with different SMA types and numbers of SMN2 copies were tested. Spearman´s rho correlations among oral function measures themselves as well as with established clinical outcome scales were analyzed.

resultsThe absolute maximum measures of oral function (maximum bite force, maximum tongue pressure, maximum mouth opening) were able to discriminate between individuals with different SMA types, individuals with a different number of SMN2 copies and with different walking abilities. The pairwise correlations of the absolute maximum measures of oral function were fair to moderate in size; the same was true for their correlations with the established motor scores. All correlations assessing endurance measures of oral function were weaker and statistically insignificant.

conclusionsAmong the oral function tests maximum tongue pressure and maximum mouth opening are particulary promising as clinical and sensitive outcome measures for clinical trials. Oral function tests may supplement existing motor scores, in particular concerning specific questions about bulbar function or in severely affected non-ambulatory individuals where mild (treatment-related) changes would otherwise remain undetected. Trial registration DRKS, DRKS00015842. Registered 30 July 2019, https://drks.de/search/de/trial/DRKS00015842.

Indexed as

Muscular Atrophy, SpinalSpinal Muscular Atrophies of ChildhoodAdultHumansOutcome Assessment, Health CarePressureTongueBite forceBulbar neuromuscular functionEnduranceFatigabilityFatigueOutcome measuresSpinal muscular atrophyTongue pressureValidity

Identifiers

PMID37138365
PMCPMC10155305

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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 OpenQuestion graph.