Tongue pressure as a novel biomarker of spinal and bulbar muscular atrophy
- 21 January 2014
- journal article
- Published by Ovid Technologies (Wolters Kluwer Health) in Neurology
- Vol. 82 (3), 255-262
- https://doi.org/10.1212/wnl.0000000000000041
Abstract
Objective: This study aimed to explore the reliability and validity of tongue pressure measurement as a quantitative evaluation of swallowing function in patients with spinal and bulbar muscular atrophy (SBMA). Methods: This study enrolled 47 genetically confirmed patients with SBMA and 38 age- and sex-matched healthy controls. In both groups we measured tongue pressure using an intraoral pressure probe and assessed questionnaires that evaluated swallowing functions. We then analyzed the relationship between tongue pressure, functional scales, and the muscle weakness of other regions. Results: Levels of tongue pressure were decreased in patients with SBMA within 3 years from the onset of the disease compared to healthy controls (SBMA 15.3 ± 6.4 kPa; healthy controls 37.3 ± 9.6 kPa; p < 0.001). Test-retest analysis showed a high reliability in patients with SBMA (intraclass correlation coefficient = 0.986). Tongue pressure showed a strong correlation with bulbar-related functional scales. Decrease of tongue pressure was detected in patients who reported no subjective dysphagia, and repetition of swallowing compensated for tongue weakness in such subjects. In patients with SBMA, tongue pressure more strongly correlates with the strength of pharyngeal, neck, and upper limb musculatures than with that of the lower limbs. Conclusion: Tongue pressure measurement is reliable and reflects swallowing function in patients with SBMA. The muscle strength of the tongue appears to decrease in SBMA before the awareness of subjective dysphagia, suggesting that tongue pressure measurement is a novel biomarker of SBMA and is applicable to early-stage detection.Keywords
This publication has 31 references indexed in Scilit:
- Pilot trial of clenbuterol in spinal and bulbar muscular atrophyNeurology, 2013
- Assessment of swallowing in motor neuron disease and Asidan/SCA36 patients with new methodsJournal of the Neurological Sciences, 2013
- Stopping Alzheimer's Before It StartsScience, 2012
- Perspectives on molecular targeted therapies and clinical trials for neurodegenerative diseasesJournal of Neurology, Neurosurgery & Psychiatry, 2012
- Efficacy and safety of dutasteride in patients with spinal and bulbar muscular atrophy: a randomised placebo-controlled trialThe Lancet Neurology, 2011
- Tongue force analysis assesses motor phenotype in premanifest and symptomatic Huntington's diseaseMovement Disorders, 2010
- Validation of a swallowing disturbance questionnaire for detecting dysphagia in patients with Parkinson's diseaseMovement Disorders, 2007
- Laryngeal Penetration During Deglutition in Normal Subjects of Various AgesDysphagia, 2007
- Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patientsBrain, 2006
- Severity of X‐linked recessive bulbospinal neuronopathy correlates with size of the tandem cag repeat in androgen receptor geneAnnals of Neurology, 1992