Tongue force analysis assesses motor phenotype in premanifest and symptomatic Huntington's disease

Movement Disorders : Official Journal of the Movement Disorder Society
Ralf ReilmannHerwig W Lange

Abstract

Motor symptoms in Huntington's Disease (HD) are commonly assessed by the Unified Huntington's Disease Rating Scale-Total Motor Score (UHDRS-TMS). However, the UHDRS-TMS is limited by interrater variability, its categorical nature, and insensitivity in premanifest subjects. More objective and quantitative measures of motor phenotype may complement the use of the UHDRS-TMS as outcome measure and increase the power and sensitivity of clinical trials. Deficits in tongue protrusion are well acknowledged in HD and constitute a subitem of the UHDRS-TMS. We, therefore, investigated whether objective and quantitative assessment of tongue protrusion forces (TPF) provides measures that (1) correlate to the severity of motor phenotype detected in the UHDRS-TMS in symptomatic HD, (2) detect a motor phenotype in premanifest HD gene-carriers, and (3) exhibit a correlation to the genotype as assessed by a disease burden score (based on CAG-repeat length and age). Using a precalibrated force transducer, the ability of premanifest gene carriers (n = 15) and subjects with symptomatic HD (n = 20) to generate and maintain isometric TPF at three target force levels (0.25, 0.5, and 1.0 N) was assessed and compared with age-matched controls (n = 20) i...Continue Reading

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Citations

Jan 14, 2012·AJNR. American Journal of Neuroradiology·S J A van den BogaardR A C Roos
Dec 22, 2015·Journal of Neuroscience Methods·Sarah SchramkeRalf Reilmann
Sep 13, 2014·Movement Disorders : Official Journal of the Movement Disorder Society·Cristina SampaioRalf Reilmann
Feb 18, 2015·Movement Disorders : Official Journal of the Movement Disorder Society·Ralf ReilmannBaltazar Gomez-Mancilla
Sep 6, 2014·Expert Review of Neurotherapeutics·Masahisa KatsunoGen Sobue
Feb 13, 2020·Journal of Neurology·Heiko GaßnerZacharias Kohl
Jul 10, 2012·Current Opinion in Neurology·Ainhi D Ha, Victor S C Fung
Aug 15, 2020·Annals of Clinical and Translational Neurology·Johnny LoTravis M Cruickshank
Nov 22, 2011·Human Brain Mapping·Rachael I ScahillUNKNOWN TRACK-HD investigators
Jun 25, 2016·Journal of Huntington's Disease·Kelly L AndrzejewskiE Ray Dorsey
Sep 17, 2020·Scientific Reports·Travis CruickshankGabriel S Trajano
Nov 14, 2021·Oral Diseases·Luciana MunhozEmiko Saito Arita

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