Quantitative assessment of lingual strength in late-onset Pompe disease.
| dc.contributor.author | Jones, Harrison N | |
| dc.contributor.author | Crisp, Kelly D | |
| dc.contributor.author | Asrani, Priyanka | |
| dc.contributor.author | Sloane, Richard | |
| dc.contributor.author | Kishnani, Priya S | |
| dc.date.accessioned | 2023-05-02T13:31:23Z | |
| dc.date.available | 2023-05-02T13:31:23Z | |
| dc.date.issued | 2015-05 | |
| dc.date.updated | 2023-05-02T13:31:23Z | |
| dc.description.abstract | IntroductionSkeletal muscle is common in late-onset Pompe disease (LOPD). Recent data implicate common bulbar muscle involvement (i.e., the tongue).MethodsWe used quantitative assessment of lingual strength to retrospectively determine the frequency and severity of lingual weakness in LOPD. We additionally examined associations between lingual strength and the presence or absence of dysarthria, and dysarthria severity.ResultsQuantitative assessment revealed lingual weakness to be present in 80% of the sample. In the 24 affected patients, severity was mild in 29%, moderate in 29%, and severe in 42%. Patients with clinical dysarthria had greater lingual weakness than those without. As dysarthria severity increased, lingual strength decreased by an average of 6.82 kPa.ConclusionsThese quantitative data provide additional evidence for presence of bulbar muscle disease in patients with LOPD. Further study is necessary to determine functional effects, temporal progression, and effects of treatment. | |
| dc.identifier.issn | 0148-639X | |
| dc.identifier.issn | 1097-4598 | |
| dc.identifier.uri | ||
| dc.language | eng | |
| dc.publisher | John Wiley and Sons Inc. | |
| dc.relation.ispartof | Muscle & nerve | |
| dc.relation.isversionof | 10.1002/mus.24523 | |
| dc.subject | Tongue | |
| dc.subject | Humans | |
| dc.subject | Muscle Weakness | |
| dc.subject | Glycogen Storage Disease Type II | |
| dc.subject | Dysarthria | |
| dc.subject | Prognosis | |
| dc.subject | Severity of Illness Index | |
| dc.subject | Incidence | |
| dc.subject | Retrospective Studies | |
| dc.subject | Age of Onset | |
| dc.subject | Adult | |
| dc.subject | Aged | |
| dc.subject | Middle Aged | |
| dc.subject | Child | |
| dc.subject | Female | |
| dc.subject | Male | |
| dc.subject | Muscle Strength | |
| dc.title | Quantitative assessment of lingual strength in late-onset Pompe disease. | |
| dc.type | Journal article | |
| duke.contributor.orcid | Jones, Harrison N|0000-0002-4171-980X | |
| duke.contributor.orcid | Kishnani, Priya S|0000-0001-8251-909X | |
| pubs.begin-page | 731 | |
| pubs.end-page | 735 | |
| pubs.issue | 5 | |
| pubs.organisational-group | Duke | |
| pubs.organisational-group | School of Medicine | |
| pubs.organisational-group | Basic Science Departments | |
| pubs.organisational-group | Clinical Science Departments | |
| pubs.organisational-group | Institutes and Centers | |
| pubs.organisational-group | Molecular Genetics and Microbiology | |
| pubs.organisational-group | Pediatrics | |
| pubs.organisational-group | Pediatrics, Medical Genetics | |
| pubs.organisational-group | Duke Clinical Research Institute | |
| pubs.organisational-group | Institutes and Provost's Academic Units | |
| pubs.organisational-group | Initiatives | |
| pubs.organisational-group | Duke Innovation & Entrepreneurship | |
| pubs.organisational-group | Head and Neck Surgery & Communication Sciences | |
| pubs.publication-status | Published | |
| pubs.volume | 51 |
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