Quantitative assessment of lingual strength in late-onset Pompe disease.

Journal Article (Journal Article)

INTRODUCTION: Skeletal muscle is common in late-onset Pompe disease (LOPD). Recent data implicate common bulbar muscle involvement (i.e., the tongue). METHODS: We 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. RESULTS: Quantitative 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. CONCLUSIONS: These 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.

Full Text

Duke Authors

Cited Authors

  • Jones, HN; Crisp, KD; Asrani, P; Sloane, R; Kishnani, PS

Published Date

  • May 2015

Published In

Volume / Issue

  • 51 / 5

Start / End Page

  • 731 - 735

PubMed ID

  • 25399907

Electronic International Standard Serial Number (EISSN)

  • 1097-4598

Digital Object Identifier (DOI)

  • 10.1002/mus.24523


  • eng

Conference Location

  • United States